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: Primary intracranial rhabdomyosarcoma is rare. Twenty-one cases with dismal outcomes have been reported. We add five children with this disease treated between 1977 and 1982. Their therapy consisted of surgical resection, craniospinal irradiation, and intravenous-intrathecal chemotherapy. Two children have recovered, 21 and 67 months after diagnosis; the last is the longest survival reported in the literature. Two succumbed to tumor recurrence, and one died due to pulmonary embolism. The posterior fossa was invariably a site of tumor at presentation. Diagnosis with light microscopy can be elusive; electron microscopic and immunohistochemical evaluation are necessary to confirm the pathological condition. This is essential so that early, aggressive therapy can be instituted. These diagnostic and therapeutic techniques have led to a reevaluation of this malignancy and its prognosis.(Neurosurgery 17: 25 …