Primary rhabdomyosarcoma of the central nervous system (CNS) is rare in both adults and children (Taratuto et al. (1985) Acta Neuropathol (Berl) 66(2):98–104). The outcome in the majority of cases is poor, and many cases are associated with early mortality (Celli et al. (1998) J Neurooncol 36(3):259–267). There are very few cases reported in the literature of survival beyond 2 years after diagnosis. We report a case of primary intracranial embryonal rhabdomyosarcoma in a 5-year-old girl who was treated successfully with local radiation therapy (RT) and a combination of two different chemotherapeutic regimens. The patient is clinically well 26 months after diagnosis, with no definitive evidence of residual disease.