Abstract
Ependymomas are rare malignant neoplasms that originate from radial glial cells within the central nervous system. Within pediatric central nervous tumors, ependymomas constitute the third most common entity with the majority occurring within the posterior fossa. Over the past decade, there have been monumental strides in classifying and grading central nervous tumors, specifically ependymomas. Revised classifications now identify ependymomas by anatomic location, histopathological and genetic subgroups with varying levels of symptom presentation and disease progression. Standard care of therapy remains surgical resection with post- operative radiotherapy.