ICD-10-CM Code For Phakomatoses Not Elsewhere Classified: Group Overview

The International Classification of Diseases, Tenth Revision, Clinical Modification (ICD-10-CM) code group Q85 includes congenital neurocutaneous syndromes and related phakomatoses not classified elsewhere, covering conditions that affect skin, nervous system, eyes, and other organs. These ICD-10-CM diagnosis codes are used to document multisystem involvement, lesion type, and clinical complexity relevant to congenital neurocutaneous disorders. Accurate coding within the ICD-10-CM Q85 group supports complete clinical documentation and appropriate reimbursement.

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ICD-10-CM Q85: Phakomatoses, Not Elsewhere Classified Overview

This group includes congenital neurocutaneous syndromes characterized by developmental abnormalities of the skin, eyes, nervous system, and other organs. Phakomatoses are multisystem disorders that often present with vascular or pigmentary lesions and potential neurologic involvement. Accurate coding is important for complete clinical documentation and appropriate reimbursement for complex multispecialty care.

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ICD-10-CM Code For Phakomatoses Not Elsewhere Classified: Group Overview

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ICD-10-CM Q85: Phakomatoses, Not Elsewhere Classified Overview

When to Use

Use a code from ICD-10-CM Q85 when a congenital neurocutaneous syndrome or phakomatosis is diagnosed and is not assigned to a more specific code elsewhere in the classification. Select the code that best reflects documented organ involvement and the specific named condition when available.

ICD-10-CM Codes

11 rows

ICD-10-CM Code	Description
Q85.00	Neurofibromatosis, unspecified
Q85.01	Neurofibromatosis, type 1
Q85.02	Neurofibromatosis, type 2
Q85.03	Schwannomatosis
Q85.09	Other neurofibromatosis
Q85.1	Tuberous sclerosis
Q85.81	PTEN hamartoma tumor syndrome
Q85.82	Other Cowden syndrome
Q85.83	Von Hippel-Lindau syndrome
Q85.89	Other phakomatoses, not elsewhere classified
Q85.9	Phakomatosis, unspecified

DRG Codes

11 rows

DRG Code	Description
091	Other Disorders of Nervous System with MCC
092	Other Disorders of Nervous System with CC
093	Other Disorders of Nervous System without CC/MCC
826	Myeloproliferative Disorders or Poorly Differentiated Neoplasms with Major O.R. Procedures with MCC
827	Myeloproliferative Disorders or Poorly Differentiated Neoplasms with Major O.R. Procedures with CC
828	Myeloproliferative Disorders or Poorly Differentiated Neoplasms with Major O.R. Procedures without CC/MCC
829	Myeloproliferative Disorders or Poorly Differentiated Neoplasms with Other Procedures with CC/MCC
830	Myeloproliferative Disorders or Poorly Differentiated Neoplasms with Other Procedures without CC/MCC
843	Other Myeloproliferative Disorders or Poorly Differentiated Neoplastic Diagnoses with MCC
844	Other Myeloproliferative Disorders or Poorly Differentiated Neoplastic Diagnoses with CC
845	Other Myeloproliferative Disorders or Poorly Differentiated Neoplastic Diagnoses without CC/MCC

Typical Clinical Scenarios

A newborn is evaluated by neonatology after delivery for multiple cutaneous and ocular findings including hypopigmented macules, a facial port-wine stain, and multiple retinal hamartomas noted on ophthalmologic exam. The infant is referred for genetic testing and baseline imaging to assess for associated central nervous system involvement; a congenital phakomatosis diagnosis from this group is assigned to capture the multisystem congenital malformation. Typical codes: Q85.0, Q85.02
A 28-year-old adult presents to dermatology with long-standing multiple neurofibromas along the trunk and plexiform masses causing pain and disfigurement; prior history includes café-au-lait macules and a family history of similar lesions. The patient seeks management of chronic disease burden and surveillance for tumor progression; a code from this group is used to document the hereditary phakomatosis as the primary diagnosis driving care. Typical codes: Q85.01, Q85.09
A pediatric patient with known tuberous sclerosis is admitted to the emergency department for acute seizures and focal neurological deficits; imaging reveals subependymal giant cell astrocytomas and new renal angiomyolipomas causing hematuria. The admission documents complications of the underlying congenital disorder and need for acute neurologic and urologic interventions, so an applicable code from this group is assigned to represent the underlying phakomatosis as a comorbidity contributing to the acute presentation. Typical codes: Q85.1, Q85.81

Related Codes

Q80-CONGENITAL-ICHTHYOSIS

ICD-10-CM Code For Congenital Ichthyosis: Group Overview

Q81-EPIDERMOLYSIS-BULLOSA

ICD-10-CM Code For Epidermolysis Bullosa: Group Overview

Q82-OTHER-CONGENITAL-MALFORMATIONS-OF-SKIN

ICD-10-CM Code For Other Congenital Malformations Of Skin: Group Overview

Q84-OTHER-CONGENITAL-MALFORMATIONS-OF-INTEGUMENT

ICD-10-CM Code For Other Congenital Malformations Of Integument: Group Overview

Q86-CONGENITAL-MALFORMATION-SYNDROMES

ICD-10-CM Code For Congenital Malformation Syndromes Due To Known Exogenous Causes: Group Overview

Q87-OTHER-SPECIFIED-CONGENITAL-MALFORMATION-SYNDROMES

ICD-10-CM Code For Other Specified Congenital Malformation Syndromes Affecting Multiple Systems: Group Overview

Q89-OTHER-CONGENITAL-MALFORMATIONS

ICD-10-CM Code For Other Congenital Malformations Not Elsewhere Classified: Group Overview

Frequently Asked Questions

References

1.CPT® Codes and Descriptions. AAPC. Retrieved Aug 11, 2026.Maintains the CPT code set's official descriptions and guidelines, licensed from the American Medical Association, used as the definitional source for CPT codes on this page.
2.HCPCS Quarterly Update. Centers for Medicare & Medicaid Services (CMS). Retrieved Aug 11, 2026.CMS's quarterly release of HCPCS Level II codes and short/long descriptors, the source for HCPCS code definitions and update history.
3.ICD-10-CM Full Code List. Centers for Medicare & Medicaid Services (CMS). Retrieved Aug 11, 2026.CMS's complete ICD-10-CM diagnosis code list, used to cross-reference diagnosis codes associated with this procedure or service.

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When to Use

ICD-10-CM Codes

11 rows

ICD-10-CM Code	Description
Q85.00	Neurofibromatosis, unspecified
Q85.01	Neurofibromatosis, type 1
Q85.02	Neurofibromatosis, type 2
Q85.03	Schwannomatosis
Q85.09	Other neurofibromatosis
Q85.1	Tuberous sclerosis
Q85.81	PTEN hamartoma tumor syndrome
Q85.82	Other Cowden syndrome
Q85.83	Von Hippel-Lindau syndrome
Q85.89	Other phakomatoses, not elsewhere classified
Q85.9	Phakomatosis, unspecified

DRG Codes

11 rows

DRG Code	Description
091	Other Disorders of Nervous System with MCC
092	Other Disorders of Nervous System with CC
093	Other Disorders of Nervous System without CC/MCC
826	Myeloproliferative Disorders or Poorly Differentiated Neoplasms with Major O.R. Procedures with MCC
827	Myeloproliferative Disorders or Poorly Differentiated Neoplasms with Major O.R. Procedures with CC
828	Myeloproliferative Disorders or Poorly Differentiated Neoplasms with Major O.R. Procedures without CC/MCC
829	Myeloproliferative Disorders or Poorly Differentiated Neoplasms with Other Procedures with CC/MCC
830	Myeloproliferative Disorders or Poorly Differentiated Neoplasms with Other Procedures without CC/MCC
843	Other Myeloproliferative Disorders or Poorly Differentiated Neoplastic Diagnoses with MCC
844	Other Myeloproliferative Disorders or Poorly Differentiated Neoplastic Diagnoses with CC
845	Other Myeloproliferative Disorders or Poorly Differentiated Neoplastic Diagnoses without CC/MCC

Typical Clinical Scenarios

A newborn is evaluated by neonatology after delivery for multiple cutaneous and ocular findings including hypopigmented macules, a facial port-wine stain, and multiple retinal hamartomas noted on ophthalmologic exam. The infant is referred for genetic testing and baseline imaging to assess for associated central nervous system involvement; a congenital phakomatosis diagnosis from this group is assigned to capture the multisystem congenital malformation. Typical codes: Q85.0, Q85.02
A 28-year-old adult presents to dermatology with long-standing multiple neurofibromas along the trunk and plexiform masses causing pain and disfigurement; prior history includes café-au-lait macules and a family history of similar lesions. The patient seeks management of chronic disease burden and surveillance for tumor progression; a code from this group is used to document the hereditary phakomatosis as the primary diagnosis driving care. Typical codes: Q85.01, Q85.09
A pediatric patient with known tuberous sclerosis is admitted to the emergency department for acute seizures and focal neurological deficits; imaging reveals subependymal giant cell astrocytomas and new renal angiomyolipomas causing hematuria. The admission documents complications of the underlying congenital disorder and need for acute neurologic and urologic interventions, so an applicable code from this group is assigned to represent the underlying phakomatosis as a comorbidity contributing to the acute presentation. Typical codes: Q85.1, Q85.81

Related Codes

Q80-CONGENITAL-ICHTHYOSIS

ICD-10-CM Code For Congenital Ichthyosis: Group Overview

Q81-EPIDERMOLYSIS-BULLOSA

ICD-10-CM Code For Epidermolysis Bullosa: Group Overview

Q82-OTHER-CONGENITAL-MALFORMATIONS-OF-SKIN

ICD-10-CM Code For Other Congenital Malformations Of Skin: Group Overview

Q84-OTHER-CONGENITAL-MALFORMATIONS-OF-INTEGUMENT

ICD-10-CM Code For Other Congenital Malformations Of Integument: Group Overview

Q86-CONGENITAL-MALFORMATION-SYNDROMES

ICD-10-CM Code For Congenital Malformation Syndromes Due To Known Exogenous Causes: Group Overview

Q87-OTHER-SPECIFIED-CONGENITAL-MALFORMATION-SYNDROMES

ICD-10-CM Code For Other Specified Congenital Malformation Syndromes Affecting Multiple Systems: Group Overview

Q89-OTHER-CONGENITAL-MALFORMATIONS

ICD-10-CM Code For Other Congenital Malformations Not Elsewhere Classified: Group Overview

Frequently Asked Questions

References

1.CPT® Codes and Descriptions. AAPC. Retrieved Aug 11, 2026.Maintains the CPT code set's official descriptions and guidelines, licensed from the American Medical Association, used as the definitional source for CPT codes on this page.
2.HCPCS Quarterly Update. Centers for Medicare & Medicaid Services (CMS). Retrieved Aug 11, 2026.CMS's quarterly release of HCPCS Level II codes and short/long descriptors, the source for HCPCS code definitions and update history.
3.ICD-10-CM Full Code List. Centers for Medicare & Medicaid Services (CMS). Retrieved Aug 11, 2026.CMS's complete ICD-10-CM diagnosis code list, used to cross-reference diagnosis codes associated with this procedure or service.

Published by

Trek Health powers OpenPayer with a leading commercial payer intelligence platform, seamlessly converting fragmented payer data into actionable insights for provider organizations.

ICD-10-CM Code For Phakomatoses Not Elsewhere Classified: Group Overview

Sign up for ICD-10-CM Code For Phakomatoses Not Elsewhere Classified: Group Overview policy alerts

ICD-10-CM Q85: Phakomatoses, Not Elsewhere Classified Overview

ICD-10-CM Code For Phakomatoses Not Elsewhere Classified: Group Overview

Sign up for ICD-10-CM Code For Phakomatoses Not Elsewhere Classified: Group Overview policy alerts

ICD-10-CM Q85: Phakomatoses, Not Elsewhere Classified Overview

When to Use

ICD-10-CM Codes

DRG Codes

Typical Clinical Scenarios

Related Codes

Which conditions are included in ICD-10-CM code group `Q85`?

What organ systems are primarily affected by conditions in `Q85`?

Why is accurate coding within `Q85` important for documentation and reimbursement?

Which DRGs may be related to hospital cases involving `Q85` diagnoses?

How are individual codes within `Q85` specified for different neurofibromatosis types?

References

When to Use

ICD-10-CM Codes

DRG Codes

Typical Clinical Scenarios

Related Codes

Which conditions are included in ICD-10-CM code group `Q85`?

What organ systems are primarily affected by conditions in `Q85`?

Why is accurate coding within `Q85` important for documentation and reimbursement?

Which DRGs may be related to hospital cases involving `Q85` diagnoses?

How are individual codes within `Q85` specified for different neurofibromatosis types?

References