RESUMO
@#This reports a case of dysembryoplastic neuroepithelial tumor (DNET) in a 5-year old male presenting with visual hallucination and seizures. Diagnostic workup revealed a homogenous cystic tumor located in the right temporo-parietal area which was considered as a low grade glioma. The patient underwent endoscopic third ventriculostomy with complete tumor excision and biopsy, revealing NET. Findings were confirmed by immunohistochemical staining with glial fibrillary action protein (GFAP), Alcian blue and synaptophysin. DNET is a recently described intracranial tumor under the World Health Organization classification of central nervous system (CNS) tumors. It is a unique entity of neuroglial tumors with excellent prognosis. Its worldwide incidence among all neuroepithelial tumors is 1.2% in patients under 20 years and 0.2% among patients over 20 years. This is the first case of DNET in the country as well as in our institution. Key points on the clinical manifestation, approach to diagnosis, distinctive radiologic and histopathologic characteristics, and management are discussed.