Persistent Mullerian duct syndrome is a rare form of pseudohermaphroditism. This article is made of two case reports showing malignant transformation. The first case was a 36-year-old male who presented with infertility. Unilateral inguinal mass and undescended testis were found on physical examination. The inguinal ultrasonography reported an inguinal mass on the same side while testicular tumor markers were within normal range. After surgical excision, pathologic examination revealed that the inguinal mass was uterus and intratubular germ cell neoplasm was present in the testis tissue. The second case was a 31-year-old male with 18 cm intraabdominal mass which was noticed during umbilical hernia repair. Physical examination revealed bilateral undescended testes with increased human chorionic gonadotropin levels. Preoperative abdominal imaging revealed a uterus posteriorly of the mass. Excision of the mass and retroperitoneal lymph node dissection were performed after neoadjuvant chemotherapy. Pathology results revealed seminoma of the testis.