Darier’s disease is an uncommon skin disorder with autosomal dominant transmission. During the course of the investigation of a large family in which several members are affected with schizophrenia, it was found that many family members also suffer from Darier’s disease. Recent advances in molecular genetic techniques have made the identification of such families important for an understanding of the causes of inherited diseases. The role of genetic linkage analysis of Darier’s disease is discussed following a review of the current state of knowledge of its pathogenesis and genetics.