Abstract
Schwannoma originates from the myelin sheath of peripheral nerves. It accounts for about 8% of all intracranial tumors. Commonly, schwannoma is located in the extra-axial locations; intra-axial schwannomas are extremely uncommon. The schwannoma arising from fourth ventricle is extremely uncommon and authors in a detailed PubMed and MEDLINE search could find only seven cases reported in the literature in the form of isolated cases report, who were managed surgically, with only one being a pediatric case. Authors report second case of intra-fourth ventricular schwannoma occurring in the pediatric age group. The hypothesis of intraventricular schwannoma is postulated to be aberrantly placed multipotent cell during embryogenesis and later transforming into Schwann cell and producing schwannoma. Pertinent literature is reviewed along with diagnosis, and management of such rare case is discussed briefly.