Snapshot An 8-year-old boy presents to the pediatric emergency department with headache, nausea, and vomiting. His symptoms progressively developed over the course of days. Physical examination is notable for a left sixth nerve palsy and papilledema. MRI brain with and without contrast demonstrates a mass in the fourth ventricle that enhances with gadolinium. Introduction Overview neoplasm arising from or adjacent to the ependymal cells that line the ventricular system accounts for < 10% of central nervous system tumors most commonly occur in the fourth ventricle or intramedullary spinal cord Epidemiology Incidence most common in early childhood though can occur at any age spinal ependymomas are more common in adults Presentation Symptoms/physical exam clinical manifestations depend on the location of the tumor most occur in the fourth ventricle, leading to hydrocephalus headache, nausea, and vomiting papilledema seizures or focal neurological deficits Imaging MRI brain with and without gadolinium indication imaging study of choice findings enhancing mass in the ventricular system may find enlarge ventricles due to hydrocephalus Studies Histopathology indication required to confirm the diagnosis and determine if the meningioma is benign or malignant findings perivascular pseudorosettes basal ciliary bodies near cellular nuclei Differential Other malignant brain neoplasm differentiating factors based on histological findings e.g., glioblastomas will have pleomorphic tumor cells surrounding a central area of necrosis Treatment Medical chemotherapy indication in very young patients bulky residual disease recurrent or refractory tumors radiation therapy indication adjuvant therapy with surgical resection in most cases Surgical resection indication mainstay of treatment Complications Seizures Hydrocephalus Prognosis Poor