Q00.0 Anencephaly
The ICD-10-CM code for Anencephaly is Q00.0 (FY2026). It is a billable, claim-ready diagnosis code.
Classification
- Section
- Q00-Q07: Congenital malformations of the nervous system (Q00-Q07)
- Category Q00
- 4 codes (3 billable)
- FY2026 Status
- Stable since FY2024
Also Known As
ICD-10-CM Alphabetic Index entries that lead to Q00.0:
- Amyelencephalus, amyelencephaly
- Acrania
- Acephalia, acephalism, acephalus, acephaly
- Hemianencephaly
- Hemicephalus, hemicephaly
- Anencephalus, anencephaly
- Agenesis › brain
- Absence (of) (organ or part) (complete or partial) › brain
Inclusion Terms
- Acephaly
- Acrania
- Amyelencephaly
- Hemianencephaly
- Hemicephaly
U.S. Hospital Utilization
- An estimated 330 U.S. inpatient stays in 2023 included Q00.0 among the documented diagnoses.
Source: National Inpatient Sample (NIS), Healthcare Cost and Utilization Project (HCUP), Agency for Healthcare Research and Quality, 2016–2023. National survey-weighted estimates.
Official Coding Guidelines
Assign Q00-QA1 when malformation/deformation/chromosomal abnormality documented17. 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 diagnosedCodes 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