A diagnosis of pericentral retinal dystrophy was made in 28 patients from four families, all living in North Norway. Patients from two and three generations were examined, establishing the relatively benign but progressive course of the disease. The advanced stage of pericentral retinal dystrophy could not be differentiated from the advanced stage of 'classical' retinitis pigmentosa. Three families demonstrated an autosomal dominant pattern of inheritance. In one family the mode of inheritance probably was autosomal recessive. Fluorescein angiography in the initial stage of the disease was normal, without evidence of pathological changes in the pigment epithelium or choroidal vessels, indicating that the pathological process may start in the photoreceptors.