Dysembryoplastic neuroepithelial tumor (DNT)- like lesions arise in extracortical locations and behave in a benign fashion similar to that of cortical DNTs. They usually come to attention by symptoms and signs of increased intracranial pressure rather than focal neurological impairment. Here we report a case of 9-year-old boy with a complaint of headache and back pain. A third ventricular mass lesion with disseminated spinal tumor was detected on his magnetic resonance imaging. The presence of floating neurons in a mucinous matrix, oligodendrocyte-like cells (OLCs) aligning axonal columns and vessels, immunohistochemical profile of the neoplasm in addition to the clinical and radiological manifestations of the patient led to the diagnosis of “DNT-like neoplasm of the third ventricle”.