A new early onset hereditary retinal degeneration is characterized in Norwegian elkhound dogs. This disease, termed early retinal degeneration (erd), was studied in 10 affected dogs, from 30 days- to 7 years old, clinically, by electroretinography, and by light- and electron-microscopic morphology. Control studies were performed on 49 non-affected dogs. Affected dogs are initially nightblind, and become totally blind between 12- and 18 months of age. The postnatal development of their rod and cone photoreceptors is abnormal both structurally and functionally. Morphologically, rod and cone inner- and outer-segment growth occurs but appears uncoordinated. Adjacent rods become very disparate in the size and proportions of their inner- and outer segments. Prominent villiform processes extend from the inner segments of rods and, to a lesser extent, cones. Synaptic terminals of rods and cones fail to develop properly. The b-wave of the electroretinogram fails to develop and the electroretinogram (ERG) remains a-wave-dominated. Subsequent to these abnormalities of development, the rods and cones degenerate, rapidly at first and later more gradually. In normal dogs, development of the ERG a- and b-waves is shown to follow, respectively, morphologic development of the photoreceptor outer segments and synaptic terminals. Similarly the abnormal development and subsequent degeneration of photoreceptor outer segments and synaptic terminals in affected dogs, correspond in time course to development and degeneration of the ERG a- and b-waves.