Four eyes of three patients from two large families with dominant exudative vitreoretinopathy showed an elevated retinal fold extending from the posterior pole toward the inferotemporal periphery of the fundus. A congenital retinal fold (ablatio falciformis congenita) has to be considered as a sign rather than a diagnosis, and can be caused by a few underlying disorders, to which dominant exudative vitreoretinopathy can be added. There are no indications that the hyaloid system is involved in the pathogenesis of a retinal fold in the latter condition. The formation of a retinal fold in dominant exudative vitreoretinopathy is the consequence of a developmental disorder of retinal vasculature during the last few months of intrauterine life, and may even occur after birth. The clinical picture and familial occurrence of many cases of congenital retinal fold described in the literature are suggestive of dominant exudative vitreoretinopathy, which only recently has been recognized as being a rather common condition.