Involvement of the retina in cases of tuberous sclerosis escaped notice until 1921, when van der Hoeve,1 of Amsterdam, Netherlands, published a clinical report describing six cases of tuberous sclerosis in which tumors of the retina were observed through the ophthalmoscope. Since then a number of clinical reports of this condition have appeared, indicating that such retinal tumors are not uncommon and that distrubance of vision and the ophthalmoscopic examination may give the first clue as to the disease. The case presented here is the sixth in which microscopic study of the retinal tumor is described and the first to be reported in this country in which such a study was made. REVIEW OF THE LITERATURE The term tuberous sclerosis, or tuberose sclerosis, was first used by Bourneville, who, in a series of articles published from 1880 to 1898, described a rare form of cerebral sclerosis observed at