The ocular clinicopathologic features of this unique patient were congenital stationary night blindness, drusen of the optic nerve head, cilioretinal artery, intraretinal pigmented lesion, and branch retinal vein occlusion. Photocoagulation therapy led to total disappearance of the neovascular tissue, clinically and histopathologically. Histopathologic examination showed an occluded branch vein associated with a sclerotic retinal arteriole. Peripheral to the site of venous occlusion, inner ischemic retinal atrophy was present. The normal complement of rod and cone photoreceptors supports the view that the night blindness in this case was an abnormality in the neural transmission and not on a morphological basis. The pigmented intraretinal lesion proved to be a localized area of retinal and choroidal neovascularization with anastomosis and secondary retinal pigment epithelial hyperplasia. This lesion was identical to Fuchs' dot of myopia but out patient was hyperopic.