Dysembryoplastic neuroepithelial tumour (DNT) is located in the cerebral cortex with very few exceptions. In this article, an extremely rare case of intraventricular DNT originating from the septum pellucidum is reported. A 25-year-old woman presented with 5-month history of headache. Cranial magnetic resonance imaging (MRI) scans revealed a mass in the right lateral and third ventricle which was hypointense on T1-weighted image, and hyperintense on T2-weighted images. No contrast enhancement was detected. The lesion was excised totally using a transcallosal-transventricular approach. Immunohistochemical examination revealed DNT. The patient was discharged without any neurological deficits. Intraventricular DNT presents with symptoms of increased intracranial pressure rather than seizures. Distinguishing DNT from other intraventricular tumours is essential as DNT is characterized by benign clinical course and does not require adjuvant therapy.