Ependymomas are neuroepithelial tumors arising throughout all compartments of the central nervous system involving the intracranial and spine, and predominantly occur in children.The new genomic profile system led to a more precise evaluation for the treatment and prognosis of ependymoma.The low sensitivity of ependymoma to adjuvant therapy is one of the important reasons for the poor prognosis of children.This article reviews the classification, diagnosis, and treatment strategies of childhood ependymoma in recent years.