Q00 Anencephaly and similar malformations

The ICD-10-CM code for Anencephaly and similar malformations is Q00 (FY2026). It is a non-billable category code — select a more specific code below for claims.

Classification

Chapter

17: Congenital malformations, deformations and chromosomal abnormalities (Q00-QA0)

Section

Q00-Q07: Congenital malformations of the nervous system (Q00-Q07)

Category Q00

4 codes (3 billable)

FY2026 Status

Stable since FY2024

Official Coding Guidelines

Assign Q00-QA1 when malformation/deformation/chromosomal abnormality documented

17. Chapter 17: Congenital malformations, deformations, and chromosomal abnormalities (Q00-QA1) Assign an appropriate code(s) from categories Q00-QA1, Congenital malformations, deformations, and chromosomal abnormalities when a malformation/deformation or chromosomal abnormality is documented. A malformation/deformation/or chromosomal abnormality may be the principal/first-listed diagnosis on a record or a secondary diagnosis.

Chapter 17 codes usable throughout life; assign Q00-QA1 when diagnosed

Codes from Chapter 17 may be used throughout the life of the patient. If a congenital malformation or deformity has been corrected, a personal history code should be used to identify the history of the malformation or deformity. Although present at birth, a malformation/deformation/or chromosomal abnormality may not be identified until later in life. Whenever the condition is diagnosed by the provider, it is appropriate to assign a code from codes Q00-QA1.

Source: CMS — ICD-10-CM Official Guidelines for Coding and Reporting, FY2026

References

• CMS — ICD-10-CM Official Code Set and Guidelines, FY2026
• CDC/NCHS — ICD-10-CM Files and Browser Tool

Cite This Page

Reference Q00 with the canonical source and edition:

Q00 — Anencephaly and similar malformations. ICD-10 Coding Pro (ICD-10-CM FY2026). https://icd10codingpro.com/codes/Q00

Canonical URL: https://icd10codingpro.com/codes/Q00