Epidermoid cysts constitute congenital, benign and rare lesions, corresponding to 0.2% to 1.8% of all intracranial tumors. Only 5% of the cases are located in the fourth ventricle. Despite their genesis in intrauterine life, they are usually diagnosed between the third and fifth decades of life due to their very slow growth pattern. The image weighted by the diffusion of the magnetic resonance is essential to establish the diagnosis. The ideal treatment consists of emptying the cystic content with complete capsule resection. In the present work, we report the case of a 31-year-old female with cerebellar syndrome that evolved with intracranial hypertension. The symptomatology was due to an obstructive hydrocephalus by an epidermoid cyst located inside the fourth ventricle, which was confirmed by the pathological anatomy.