Fifteen patients in three families with familial exudative vitreoretinopathy were examined and followed. Eleven of the 15 (73%) were asymptomatic and showed only peripheral non-vascularization of the peripheral temporal retina. An acute proliferative form resembling acute retrolental fibroplasia was characterized by dragging of the retina to the temporal side and subretinal exudation which developed in children between the ages of birth and ten years. The acute proliferative changes in pediatric patients typically occurred asymmetrically, with one eye remaining asymptomatic or less severely affected while the other eye progressed to severe temporal dragging of the retina, subretinal exudation, and retinal detachment.