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 duct derivatives in an otherwise normally virilized male with a 46, XY karyotype. We discuss a rare presentation of this disorder, bilateral gonadal tumours with obstructive uropathy, and its management, together with a review of the literature.