Intracranial epidermoid cysts are one of the rare tumors of all intracranial tumors. They represent 0,2 to 1% of intracranial tumors and 7% of tumors in the cerebellopontine angle. The pineal region is exceptionally subject to such kind of tumor. Cushing was the first to report the pineal localization of the epidermoid cyst in 1928. Up to now, 85 cases of pineal epidermoid cyst were cited in the literature. We report a clinical case concerning a 45 years old man who presented an intracranial hypertension during 18 months. The clinical examination found a hemiparesis with a facial hypoesthesis. The MRI showed a process of the pineal region. The patient underwent a surgery with a large resection. The histological examination confirms the epidermoid cyst. Many approaches were described in the literature. The outcome is related to this localization.