BACKGROUND Epidermoids of the brain are rare tumors. Their fourth ventricle localization is about the rarest localization. No series of such tumors has been reported. Their manifestations and management are therefore not well understood. METHODS Four epidermoid tumors of the fourth ventricle were collected over a period of 39 years from the neurosurgical units of the American University of Beirut Medical Center and the Orient Hospital. These were retrospectively studied and the medical literature reviewed. RESULTS Epidermoids of the brain form 1% of all brain tumors, and those located in the fourth ventricle are 16.7% of the epidermoids. They rarely present symptoms of increased intracranial pressure. They commonly present in adults with only cerebellar signs. The duration of symptoms may be short, medium, or long. The diagnosis, when clinically suspected, should be confirmed by magnetic resonance imaging. An early diagnosis is the key for a good outcome. CONCLUSIONS Although a rare condition, these tumors can be lethal if not treated. Total excision should be attempted. Because this tumor is very slow-growing, a near-total excision is acceptable in cases where complete removal may endanger function or life.