We herein report a sporadic case of Darier's disease localized to the bilateral side of the neck in a 39-year-old Japanese woman. Several clinical variants of Darier's disease have so far been recognized including unilateral Darier's disease, localized Darier's disease, segmental Darier's disease and acral Darier's disease. Few cases of Darier's disease, restricted to sun-exposed areas such as the bilateral side of the neck, have been described in the published work. It remains controversial, however, whether ultraviolet exposure can induce the onset of Darier's disease. Our patient's skin lesions, which were resistant to previous treatment with corticosteroid, improved substantially with high-concentration tacalcitol lotion and sunscreen. This is the first report on the efficacy of topical tacalcitol lotion associated with sunscreen for the treatment of localized Darier's disease.