Male pseudohermaphrodite reared as a female.

A 31-year-old male pseudohermaphrodite has been brought up as a female. The parents had told their child not to discuss the problem of ambiguous genitalia with anyone. As a teenager masculinization had occurred. Chromosome analysis of both peripheral blood and gonadal tissue was 46XY. The internal gonads were testes surrounded by a rim of ovarian stroma. A rudimentary cervix opened into a small vagina, as did the urethra in a valve-like fashion. The phallus was amputated during surgical reconstruction of the external genitalia to create a phenotypic female and the vagina was enlarged. Oestrogen therapy resulted in breast development.