Ependymomas are uncommon neoplasms of the central nervous system (CNS), and as a consequence, few randomized, clinical trials have been performed, thereby limiting treatment guidelines. A review of the literature would permit the following conclusions regarding treatment. The best management of newly diagnosed ependymoma entails a complete resection corroborated by postoperative contrast-enhanced magnetic resonance imaging (MRI). If an incomplete resection is documented, a second attempt at gross total resection should be considered, given the prognostic significance of complete resection. Small volume residual disease is best managed with involved-field radiotherapy unless postoperative staging (cerebrospinal fluid cytology, neuraxis MRI) documents metastatic disease, which is best managed by craniospinal irradiation. The role of chemotherapy is uncertain and in general would be reserved for patients having previously failed surgery and radiotherapy. Disease-free survival following recurrence is unusual (<15% at 5 years) and suggests intensification of initial adjuvant treatment may best prevent relapse.