The temporal bone is the primary site for approximately 10% of rhabdomyosarcomas of the head and neck in children. Until recently it has been a uniformly fatal tumor despite treatment with radical surgery and radiation therapy. Although experience with the Intergroup Rhabdomyosarcoma Study protocol-II is of relatively short duration, treatment of these children with radiation therapy and multiple-drug intravenous and intrathecal chemotherapy holds promise of significantly improved control rates.