Object An intracranial ependymoma is a relatively rare but very interesting variety of glioma. In this paper, the authors compiled a review of the pathological features, imaging characteristics, and treatment strategies related to this brain tumor. Methods A Medline search was conducted using the term “ependymoma.” The bibliographies of papers obtained were also checked for articles and chapters that could provide additional understanding of this tumor. Malignant ependy-momas and ependymomas of the spinal cord (including myxopapillary ependymomas) were excluded from this review. Conclusions The posterior fossa is the most frequent site for an intracranial ependymoma. Children are frequently affected. Most authors recommend resecting as much of the tumor as is safely possible. Microscopically, ependymal tumors show both epithelial and glial features. Glial fibrillary acidic protein immunohistochemistry, therefore, helps in identifying ependymomas. Because ependymomas often recur despite surgical intervention, radiotherapy and/or radio-surgery may also play an important role in their treatment. The use of chemotherapy in the treatment of these tumors, especially in the very young, is still being studied.