Darier's disease (DD) is an autosomal-dominant skin disorder characterized by loss of adhesion between epidermal cells and abnormal keratinization. Several patients with unilateral, linear, 'zosteriform' or localized lesions have been reported. We report three cases of DD in a localized pattern corresponding to mosaicism type 1 according to Happle's classification and review the literature about the genetic cause of DD and DD in a 'zosteriform' pattern.