Female pseudohermaphroditism, not elsewhere classified

This code describes a condition where an individual with XX chromosomes (genetically female) presents with masculinized external genitalia, but possesses normal female internal reproductive organs (ovaries, uterus). This virilization is typically due to excessive androgen exposure during fetal development, often from congenital adrenal hyperplasia (CAH).

Use this code for patients diagnosed with female pseudohermaphroditism where the underlying cause is not specifically classified elsewhere. This applies when documentation confirms XX karyotype, ovaries and uterus, and external genitalia that are ambiguous or virilized. Supporting documentation should include genetic testing results, imaging of internal organs, and clinical examination findings.

• Do not use this code for true hermaphroditism (presence of both ovarian and testicular tissue) or male pseudohermaphroditism.
• Always code the underlying cause, such as congenital adrenal hyperplasia (E25.0), as a primary diagnosis when known.
• Ensure documentation clearly distinguishes between ambiguous genitalia (Q56.4) and pseudohermaphroditism.