[Persistent Müllerian ducts syndrome].

Persistent Müllerian ducts syndrome is a rare form of internal male pseudohermaphroditism, characterized by the presence of the uterus, fallopian tubes and upper vagina in an otherwise normally virilised male with a 46 XY karyotype. It is the result of a deficiency in anti-Müllerian hormone or abnormality of this hormone receptor. Often, the diagnosis is made incidentally during surgical exploration for cryptorchidism or herniorrhaphy, and exceptionally during cancer of ectopic testis. We discuss a rare case of this syndrome revealed by a bilateral intra-abdominal gonadal tumor.