2021 Volume 57 Issue 7 Pages 1122-1126
A one-year and five-month-old boy was referred to our hospital owing to suspected undescended testis. His left testis was not palpable at the age of four months. He had normal male-type external genitalia. The right testis was palpable in the right scrotum, but the left testis was not in the left inguinal region, which we suspected to be left intraperitoneal testis on the basis of ultrasonography showing a small mass existing above the left internal inguinal ring. We performed laparoscopy for intraperitoneal scrutiny and orchiopexy. The left inguinal mass located in the inguinal duct was considered an atrophic testis and was removed with an anterior approach. Orchiopexy was performed on the right testis to prevent torsion. No other abnormalities were found in the abdominal cavity. Histopathological examination revealed no testicular tissue in the left inguinal mass, but oviduct, ovary, and endometrial tissues were found. A chromosomal test showed 46XY. Thus, we diagnosed him as having ovotesticular disorder of sex development (DSD) because of the presence of ovarian and testicular tissues within the same individual. Our case is a rare DSD because it was not accompanied by ambiguous genitalia and was diagnosed in early childhood.