Epidermal nevi are harmatormas that are the result of developmental malformations of the epidermis, and these lesions present with a variety of clinical and histological characteristics. Some tumors can arise in epidermal nevi because epidermal nevi develop from multipotent germinative cells. We report here on a 57-year-old woman who had epidermal nevi arranged in a linear pattern since childhood. Her epidermal nevi were located in her left chest, arm and shoulder, upon which multiple trichillemal cysts seemed to develop after several years. We think that this patient with linear epidermal nevi associated with multiple trichilemmal cysts is a rare and distinctive case. (Korean J Dermatol 2009;47(1):55~58)