Persistent müllerian duct syndrome.

Persistent müllerian duct syndrome is a rare form of male pseudohermaphroditism. It is usually diagnosed in the early years of life. A case is reported of normal male appearance with bilateral undescended testicles and a right inguinal hernia. On exploration a uterus, two fallopian tubes and a testis were found in the hernial sac. As these patients have an increased risk of gonadal tumours they should be kept under long-term review.