Ependymomas are glial neoplasms arising from the ependymal lining of the ventricle or central canal of spinal cord. These tumors account for 8–10% of central nervous system tumors in children. Although the pediatric ependymomas can occur both in brain and spine, the latter is less frequently seen; approximately 90% of childhood ependymomas are present in the intracranial compartment. The World Health Organization (WHO) 2021 recently classifies the ependymal tumors, depending on their site of origin into supratentorial, posterior fossa, and spinal types which are further characterized according to their molecular profile [1]. Based on histological grade, the ependymomas have been conventionally categorized into grade 1 (subependymoma), grade II (classic), and grade III (anaplastic) variety, albeit the tumor determined outcomes with regard to their histological grade alone have been inconsistent.