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    v1.0.28
    OpenPayerBilling CodesOther congenital malformationsICD-10-CM q85-phakomatoses-not-elsewhere-classified
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    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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    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 CodeDescription
    Q85.00Neurofibromatosis, unspecified
    Q85.01Neurofibromatosis, type 1
    Q85.02Neurofibromatosis, type 2
    Q85.03Schwannomatosis
    Q85.09Other neurofibromatosis
    Q85.1Tuberous sclerosis
    Q85.81PTEN hamartoma tumor syndrome
    Q85.82Other Cowden syndrome
    Q85.83Von Hippel-Lindau syndrome
    Q85.89Other phakomatoses, not elsewhere classified
    Q85.9Phakomatosis, unspecified

    DRG Codes

    11 rows

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