The authors describe a family in which one member had pattern dystrophy of the retinal pigment epithelium. The proband also had neovascular membranes which resulted in decreased visual acuity. Two other family members had abnormal electro-oculographic findings, but a clinical normal-appearing fundus that could represent an early stage of the disease. The incidence of this condition in this family indicates autosomal dominance as the inheritance pattern of this dystrophy.