Of 24 examined family members, 14 had an undescribed macular dystrophy. There is a clear autosomal-dominant heredity. Essential features of the disease are pigmentary disturbance, cystoid macular edema, wrinkling of the internal limiting membrane, and vitreous body changes. In this family there was an increased incidence of severe hyperopia (greater than or equal to 6 diopters). As the disease progressed color vision deteriorated (type 1 acquired red-green defect), disturbance of the electro-oculogram became more prevalent, and the electroretinogram was normal. These finding differentiate this dystrophy from other diseases with a bull's eye aspect or cystoid macular edema and from vitreoretinal syndromes.