Congenital absence of vulva

This code signifies the complete congenital absence of the external female genitalia, specifically the labia majora, labia minora, clitoris, and vestibule. It represents a rare developmental anomaly present at birth, indicating a failure of these structures to form during fetal development.

Apply this code when documentation explicitly states a diagnosis of congenital absence of the vulva. This diagnosis is typically made at birth or during early childhood upon physical examination. Supporting documentation should clearly describe the lack of external female genital structures.

• Ensure documentation specifies "congenital absence" to differentiate from acquired conditions or agenesis of other genital organs.
• Do not confuse with hypoplasia or partial absence; this code is for complete absence.
• Consider sequencing with other congenital anomalies if present, following ICD-10-CM guidelines for multiple congenital malformations.