Alström's syndrome consists of pigmentary retinopathy, diabetes, obesity, normal mental capacity, and sensorineural deafness. The early retinal findings are optic atrophy, salt-and-pepper pigment epithelial abnormalities, and marked vascular attenuation. Later in the disease process, diffuse areas of chorioretinal atrophy and large clumps of pigment develop. Severe visual loss in the first decade is characteristic and is an important point in the differential diagnosis. Clinically evident renal disease is a variable age-related phenomenon, and is probably the most frequent cause of death.