[Mixed gonadal dysgenesis with plasty in males]

A 13-year-old Korean boy is reported here as a case of mixed gonadal dysgenesis. The patient presented with perineal hypospadia. He had scrotal testis, epididymis, vas deferens on right side, and abdominal streak gonad, Fallopian tube on left side. He had also uterus and vagina. His chromosome was 46XY type. Among 59 reported cases in Japan, 12 had undergone plasty into male. All of them had hypospadia. In 51 cases, 17 had testes in scrotum. None of them had a testicular tumor. So we decided to do plasty in male, and to follow up his testis quite carefully.