Most childhood rhabdoid tumors occur in the kidney or central nervous system but they can occur in other sites and they usually run an aggressive clinical course. We report a case of an 8-month-old boy with a right temporal bone rhabdoid tumor treated with surgery, chemotherapy and Gamma Knife-based stereotactic radiosurgery. The patient remained alive after 61 months and repeat magnetic resonance imaging (MRI) of the brain showed no evidence of recurrence. There were no obvious endocrine deficits or growth abnormalities at last follow-up. Gamma Knife-based stereotactic radiosurgery may have a role in the management of very young children with skull base tumors.