Intracranial epidermoid tumours are very rare lesions of ectodermal origin that account for 1% of all intracranial tumours. The favourite sites affected by these tumours are the cerebellopontine angle and the chiasmal region. They also appear in the cerebral hemisphere and the intraventricular cavities. We report a case of epidermoid tumour of the third ventricle. The patient was a 68 year-old woman admitted in our service with a 7-days history of headaches and intermittent nausea and vomiting. A CT scan of the brain revealed a large hypodense mass in the third ventricle associated with hydrocephalus. An MRI was performed and revealed, on T1-weighted image, an irregular nonhomogeneous lower signal in relation with CSF. This tumour showed a very bright signal intensity on T2-weighted images. A transcallosal approach of the tumour mass was performed via a right frontal craniotomy. Using an ultrasonic aspirator, the tumour was removed. In a review of the literature, we found only 8 reported cases.