Persistent Mullerian duct syndrome presenting with bilateral intra-abdominal gonadal tumours and obstructive uropathy

Persistent Mullerian duct syndrome is a rare, autosomal recessive intersex disorder characterized by the presence of completely developed Mullerian du ct derivatives in an otherwise normally virilized mate with a 46, XY karyot ype. We discuss a rare presentation of this disorder, bilateral gonadal tum ours with obstructive uropathy, and its management, together,with a review of the literature.