Ependymal tumors of the CNS arise from the ependymal cells that line the ventricles of the brain or the central canal of the spinal cord and can affect any age group, although they are more common in children. Approximately 60% of ependymal tumors occur in the cerebral hemispheres, 30% in the posterior fossa, and 10% in the spinal canal. The two slow-growing grade I tumors are subependymoma and myxopapillary ependymoma, whereas more aggressive-grade tumors are ependymoma (grade II) and anaplastic ependymoma (grade III). Anaplastic ependymomas are more common in posterior fossa in children and supratentorial brain in adults. There are three rare distinct histological variants—papillary, clear cell, and tanycytic ependymomas. A recently defined distinct molecular subtype of ependymoma, the RELA fusion-positive, is an aggressive variant (grade II or III) of ependymoma occurring in the supratentorial brain in children and carries a worse prognosis. Although historically considered a single entity, ependymomas from each of the three anatomic compartments (supratentorial, posterior fossa, spinal canal) are considered biologically and genetically distinct entities.