The persistent mu ̈llerian duct syndrome is a rare form of
male intersex disorder in which a phenotypically normal
male subject has fallopian tubes and a uterus along with
testes and wolffian duct derivatives. This internal male
pseudohermaphroditism is caused by a deficiency of mu ̈llerian
duct inhibitory factor.1 A genetic mutation of chromosome
19 seems to be implicated in this condition.2 Like other
undescended testes these gonads are at increased risk of
malignant transformation, and a variety of germ cell tumors
have been reported in association with this syndrome.3 These
patients often present with cryptorchidism or an inguinal
hernia with fallopian tubes and a uterus. We report a case of
bilateral synchronous germ cell tumor in a patient with the
persistent mu ̈llerian duct syndrome.
