The treatment of bladder and prostate rhabdomyosarcoma (RMS) is highly controversial. Aside from chemotherapy, treatment modalities include conservative surgery, radical surgery, and pre-, intra-, or postoperative irradiation. Between 1968 and 1993, 78 children with RMS were treated at our institution. In all, 22 tumors were located in the urogenital tract (bladder/prostate, 13; paratesticular, 5; vaginal, 2; others, 2). Altogether, 6 patients had stage II disease; 7, stage III disease; and 2, stage IV disease. All 15 patients with RMS of the bladder, prostate, or vagina received chemotherapy, and 4 had additional radiotherapy. Surgery was also done in 10 patients; parents refused an operation in 3 cases. In all, 3 patients in an advanced tumor stage died of their disease. All other children currently show no evidence of disease (mean follow-up, 6 years; range, from 2 months to 18 years). After chemotherapy, radical operative intervention with multiple biopsies (encircling the tumor)--in contrast to local tumor excision or partial resection--permits complete tumor resection followed by excellent long-term results. Following radiotherapy, often a consequence of organ-sparing therapy, many complications ensued (60%); therefore, irradiation should be restricted to highly selected cases.