Epidermoid cyst of the brain is a rare benign embryonal tumor. It has an excellent prognosis because of its slow-growing nature. Clinically, symptoms are variable and depend on the location of the cyst. Sudden death may occur as a result of lethal complications because of the tumor growth, but it is still rare. In this article, we present a rare case of sudden unexpected death of a 58-year-old man, with no neurologic history, due to an epidermoid cyst of the brain diagnosed at autopsy.