A congenital retinal fold is an anomaly characterized by a fold of retina which projects into the vitreous. It usually extends from the optic disk to the ora serrata. Microscopic studies show imperfect development of the layers of the retina within and without the fold. In the case to be reported there were congenital retinal folds similar to those described by others. In addition, the patient had microcephaly, microcrania and subnormal mentality. As far as I could determine, no other case has been reported with this combination of defects. Thirty-eight cases of congenital retinal folds have been described in the literature. Cases belonging to this group were described under other titles by Sulzer1 in 1888, Salffner2 in 1902, Heine3 in 1904, DeVries4 in 1908, Holm5 in 1923, Anacona6 in 1935 and Hoffmann7 in 1926. In 1928, Mann8 described the condition as a