Absence of antimuellerian hormone or defective functioning of antimullerian hormone type II receptors give rise to a type of male pseudohermaphroditism known as 'persistent muellerian duct syndrome'. Such patients are phenotypically male with 46 XY karyotype and well developed muellerian duct derivatives which may be intra-abdominal or may herniate in inguinal region giving rise to a condition called as 'hernia uterine inguinale'. Cryptorchidism, transverse testicular ectopia and hypospadias are few anomalies associated with this syndrome. Though orchidopexy while leaving the uterus and fallopian tube in situ is an accepted surgical management, compromisation of blood surply to the testes or the vas may occur as vasa efferentia are embedded in the wall of uterus.