The report describes a Finnish family in which retinal lesions associated with a considerable visual loss have been found in 19 probands in 5 consecutive generations. The progressive cone-rod dystrophy diagnosed in the probands shows an autosomal dominant mode of inheritance. The onset of the disease was noticed in most of the probands early during the first decade of life. In the young diseased probands the fundal lesions showed the pattern found in pure cone dystrophies. Elder probands, however, had lesions and dysfunctions indicating an obvious rod involvement in addition to the cone dystrophic pattern. The fundal lesions included very extensive pigmentation in most of the elder probands with the disease. In addition to the retinal lesions, considerable astigmatism and lens opacities at the level of the posterior capsule were found in a great proportion of the probands.