Genitourinary rhabdomyosarcoma (RMS) accounts for approximately 25% of all rhabdomyosarcomas. Management of RMS at this site has changed during the last 5 consecutive Intergroup Rhabdomyosarcoma (IRS) trials, with increasing emphasis of bladder and vaginal conservation. As more effective treatment regimens has improved survival, surgical approaches have evolved to less aggressive management of the primary tumour to improve conservation. Various combinations of chemotherapy, irradiation and surgery have resulted in a decreased late sequelae in the group of patients with sarcoma arising in the genitourinary tract.