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

The International Classification of Diseases, Tenth Revision, Clinical Modification (ICD-10-CM) code group Q07 includes congenital malformations of the nervous system such as specified and unspecified structural abnormalities of the brain, spinal cord, and related neural tissues that are present at birth. These ICD-10-CM diagnosis codes are used to document clinical scenarios involving congenital neurologic defects, capturing disease severity, anatomic involvement, and clinical complexity relevant to surgical, medical, or therapeutic management. Accurate coding within the ICD-10-CM Q07 group supports proper claim adjudication and complete clinical documentation and appropriate reimbursement.

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ICD-10-CM Q07: Other Congenital Malformations Of Nervous System Overview

The ICD-10-CM Q07 group captures a set of congenital malformations affecting the central and peripheral nervous systems present at birth. These conditions include structural and developmental anomalies of the brain, spinal cord, and associated neural structures that can affect neurologic function. Accurate coding ensures complete clinical documentation and supports appropriate reimbursement for services related to congenital neurologic disorders.

When to Use

Use a code from this group when a congenital structural or developmental abnormality of the central or peripheral nervous system is documented as present at birth. Assign the most specific code available based on the documented anatomic site and any associated complications or qualifiers.

ICD-10-CM Codes

6 rows

ICD-10-CM Code	Description
Q07.00	Arnold-Chiari syndrome without spina bifida or hydrocephalus
Q07.01	Arnold-Chiari syndrome with spina bifida
Q07.02	Arnold-Chiari syndrome with hydrocephalus
Q07.03	Arnold-Chiari syndrome with spina bifida and hydrocephalus
Q07.8	Other specified congenital malformations of nervous system
Q07.9	Congenital malformation of nervous system, unspecified

DRG Codes

5 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
791	Prematurity with Major Problems
793	Full Term Neonate with Major Problems

Typical Clinical Scenarios

A neonate is delivered with macrocephaly, hypotonia, and seizures noted shortly after birth; head ultrasound and MRI demonstrate agenesis of parts of the corpus callosum with associated ventricular enlargement. The infant is admitted to the neonatal ICU for seizure control and further genetic and neurologic evaluation, and a congenital malformation of the nervous system code is assigned to capture the primary structural brain anomaly. Typical codes: Q07.01, Q07.00
A 6-year-old child with developmental delay and spastic diplegia is followed in pediatric neurology; prior imaging documented hypoplasia and focal malformation of the cerebellum contributing to coordination problems and speech delay. The visit documents ongoing care for the congenital cerebellar malformation as the underlying cause of the chronic neurologic deficits, and a specific congenital nervous system malformation code is used for problem list and care coordination. Typical codes: Q07.02, Q07.8
An adult presents to the emergency department after a fall and is found to have new-onset worsening gait instability and progressive myelopathy; review of history and prior imaging reveals a congenital malformation of the spinal cord (such as diastematomyelia or spinal dysraphism) now complicated by tethered cord requiring neurosurgical assessment. The acute presentation is coded along with the underlying congenital nervous system malformation to document the structural etiology of the current complication. Typical codes: Q07.03, Q07.9

ICD-10-CM Code For Anencephaly And Similar Malformations: Group Overview

Q01-ENCEPHALOCELE

ICD-10-CM Code For Encephalocele: Group Overview

Q02-MICROCEPHALY

ICD-10-CM Code For Microcephaly: Group Overview

Q03-CONGENITAL-HYDROCEPHALUS

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

Q04-OTHER-CONGENITAL-MALFORMATIONS-OF-BRAIN

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

Q05-SPINA-BIFIDA

ICD-10-CM Code For Spina Bifida: Group Overview

Q06-OTHER-CONGENITAL-MALFORMATIONS-OF-SPINAL-CORD

ICD-10-CM Code For Other Congenital Malformations Of Spinal Cord: 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

6 rows

ICD-10-CM Code	Description
Q07.00	Arnold-Chiari syndrome without spina bifida or hydrocephalus
Q07.01	Arnold-Chiari syndrome with spina bifida
Q07.02	Arnold-Chiari syndrome with hydrocephalus
Q07.03	Arnold-Chiari syndrome with spina bifida and hydrocephalus
Q07.8	Other specified congenital malformations of nervous system
Q07.9	Congenital malformation of nervous system, unspecified

DRG Codes

5 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
791	Prematurity with Major Problems
793	Full Term Neonate with Major Problems

Typical Clinical Scenarios

A neonate is delivered with macrocephaly, hypotonia, and seizures noted shortly after birth; head ultrasound and MRI demonstrate agenesis of parts of the corpus callosum with associated ventricular enlargement. The infant is admitted to the neonatal ICU for seizure control and further genetic and neurologic evaluation, and a congenital malformation of the nervous system code is assigned to capture the primary structural brain anomaly. Typical codes: Q07.01, Q07.00
A 6-year-old child with developmental delay and spastic diplegia is followed in pediatric neurology; prior imaging documented hypoplasia and focal malformation of the cerebellum contributing to coordination problems and speech delay. The visit documents ongoing care for the congenital cerebellar malformation as the underlying cause of the chronic neurologic deficits, and a specific congenital nervous system malformation code is used for problem list and care coordination. Typical codes: Q07.02, Q07.8
An adult presents to the emergency department after a fall and is found to have new-onset worsening gait instability and progressive myelopathy; review of history and prior imaging reveals a congenital malformation of the spinal cord (such as diastematomyelia or spinal dysraphism) now complicated by tethered cord requiring neurosurgical assessment. The acute presentation is coded along with the underlying congenital nervous system malformation to document the structural etiology of the current complication. Typical codes: Q07.03, Q07.9

ICD-10-CM Code For Anencephaly And Similar Malformations: Group Overview

Q01-ENCEPHALOCELE

ICD-10-CM Code For Encephalocele: Group Overview

Q02-MICROCEPHALY

ICD-10-CM Code For Microcephaly: Group Overview

Q03-CONGENITAL-HYDROCEPHALUS

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

Q04-OTHER-CONGENITAL-MALFORMATIONS-OF-BRAIN

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

Q05-SPINA-BIFIDA

ICD-10-CM Code For Spina Bifida: Group Overview

Q06-OTHER-CONGENITAL-MALFORMATIONS-OF-SPINAL-CORD

ICD-10-CM Code For Other Congenital Malformations Of Spinal Cord: 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 Other Congenital Malformations Of Nervous System: Group Overview

Sign up for ICD-10-CM Code For Other Congenital Malformations Of Nervous System: Group Overview policy alerts

ICD-10-CM Q07: Other Congenital Malformations Of Nervous System Overview

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

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ICD-10-CM Q07: Other Congenital Malformations Of Nervous System Overview

When to Use

ICD-10-CM Codes

DRG Codes

Typical Clinical Scenarios

Related Codes

References

When to Use

ICD-10-CM Codes

DRG Codes

Typical Clinical Scenarios

Related Codes

References

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

Sign up for ICD-10-CM Code For Other Congenital Malformations Of Nervous System: Group Overview policy alerts

ICD-10-CM Q07: Other Congenital Malformations Of Nervous System Overview

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

Sign up for ICD-10-CM Code For Other Congenital Malformations Of Nervous System: Group Overview policy alerts

ICD-10-CM Q07: Other Congenital Malformations Of Nervous System 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 `Q07`?

What specific `Q07` codes document Arnold-Chiari syndrome and its complications?

Which `Q07` codes should be used when the congenital nervous system malformation is unspecified or otherwise specified?

How does accurate coding in `Q07` affect billing and reimbursement?

What related DRGs are associated with the `Q07` diagnosis group?

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 `Q07`?

What specific `Q07` codes document Arnold-Chiari syndrome and its complications?

Which `Q07` codes should be used when the congenital nervous system malformation is unspecified or otherwise specified?

How does accurate coding in `Q07` affect billing and reimbursement?

What related DRGs are associated with the `Q07` diagnosis group?

References