Rhabdomyosarcoma of the prostate is a rare tumor and its prognosis is extremely poor. However, the survival rate has been gradually improved by using combined chemotherapy. A 17-year-old man with the chief complaint of dysuria was referred to our hospital on April 7, 1986. Prostatic needle biopsy was performed and pathological diagnosis was rhabdomyosarcoma of the prostate. The patient was given preoperative combined chemotherapy consisting of actinomycin D and cyclophosphamide. Total cystoprostaurethrectomy and ileal conduit were performed on April 30, 1986. He underwent postoperative combined chemotherapy (vincristine, actinomycin D, cyclophosphamide). He is well 13 months after his initial symptoms and is clinically free of tumor 12 months postoperatively. Forty-two cases of rhabdomyosarcoma of the prostate including our case were collected from the Japanese literature and reviewed with respect to the multimodal treatment and prognosis.