Familial persistent müllerian duct syndrome

We found persistent müllerian duct structures in 2 phenotypically normal brothers who had undergone an operation for inguinal hernia. The older boy had transverse testicular ectopia and his younger brother had unilateral cryptorchidism. Bilateral orchiopexy was done with excision of the fallopian tubes, uterus and upper vagina. Histological examination showed normal testes in both patients. Since there is a risk of testicular malignancy in such patients, long-term followup is mandatory.