Keratoacanthoma centrifugum marginatum (KCM) is a rare entity, usually classified as solitary keratoacanthoma (KA). The Ferguson-Smith type is the most common form of multiple KAs. Because development of multiple KAs and KCM in a single patient has rarely been reported, this association presents a therapeutic challenge. We report a 46-year-old man with Ferguson-Smith multiple KAs and KCM, who was successfully treated with acitretin.