A case of prune belly syndrome associated with urethral stricture and atresia ani is reported. Cystostomy and colostomy were performed on his birthday. Cutaneous vesicostomy, bilateral ureterocystoneoplasty and proctoplasty were performed when the patient was one year old, and urethrotomy was performed when he was four years old. He was closely followed for six years and six months, and had developed without any serious episodes. We collected 128 cases of prune belly syndrome from the Japanese literature including this, and made a brief discussion of this syndrome with urethral stricture and atresia ani.