Epidermoid cysts of the brain are rare tumor-like lesions, most often of maldevelopmental origin. They are benign in nature, causing symptoms depending on their localization. Surgical resection leads to excellent results. A case is reported of a 68-year-old-man who died suddenly and unexpectedly. Postmortem examination revealed signs of central dysregulation and a bifrontal epidermoid cyst. To the authors’ knowledge, death resulting from epidermoid cyst of the brain has not been reported so far.