Two families showed a retinal pigment epithelial dystrophy characterized by an X-shaped yellowish macular lesion and numerous flavimaculatus retinal flecks. Nine members were variously affected. The condition was bilateral, had a dominant inheritance and started in middle age with a slow-developing macular lesion. Visual functions were often minimally disturbed for 2 or 3 decades. The flavimaculatus flecks which differed in number appeared only as secondary phenomena yet increased in number and size. At the onset of the disease, the ERG and EOG as well as colour vision were normal and became altered only in the course of a very slow process.