Patients with keratotic lesions distributed in a unilateral, linear, zosteriform or localized pattern and revealing histopathologic features of dyskeratotic acantholysis have been reported previously. There is some controversy in the appropriate nosologic classification of this entity. Some authors consider it as a localized form of Darier’s disease while others place it as a variant of epidermal nevus. We report a 19-year-old girl who presented with 14 years history of an asymptomatic, hyperkeratotic area of skin-colored papules in a dermatomal distribution on the dorsum of her left hand and forearm. Pathological evaluation showed acantholysis and dyskeratosis. The controversy regarding the correct classification of such a patient is discussed.