Ependymoma is a relatively common pediatric primary central nervous system tumor of childhood with a variable prognosis. Supratentorial and infratentorial ependymomas differ biologically. The best outcomes have been described in patients whose tumors are amenable to gross total resection. Treatment with surgery alone is at times possible in children with totally resected supratentorial tumors. For those patients with infratentorial tumors and those with supratentorial anaplastic lesions, radiation therapy is most commonly employed. New radiation techniques, including various forms of conformal radiation, have resulted in excellent disease control. Chemotherapy may have a role in the management of anaplastic or subtotally resected tumors.