Twenty-four children with rhabdomyosarcoma of the middle (22 patients) or external ear (two patients) were entered on the Intergroup Rhabdomyosarcoma Study-I protocol from 1972 to 1978. After surgery, treatment consisted of radiotherapy plus vincristine, dactinomycin, and cyclophosphamide, with or without Adriamycin. Nine of 19 patients (47%) who presented with localized sarcoma are free of disease at 2.2 to 6.5 years after diagnosis (median, 3.6 years). One is alive with regional recurrence at 6.7 years; another developed a contralateral cerebellar astrocytoma 4.4 years from diagnosis and died without evidence of rhabdomyosarcoma 2 months later. The other 13 children died of recurrent rhabdomyosarcoma at 5 to 25 months after diagnosis (median duration of survival, 10 months). Outcome was influenced by the presence of diagnosis of signs indicating meningeal extension; death rates were 5 of 5 patients with intracranial tumor, 3 of 4 with petrous bone erosion, 2 of 6 with facial nerve palsy, and 3 of 9 with no initial evidence of meningeal extension.