Pigmentary degeneration of the retina is a familial and hereditary disease in which the peripheral portion of the retina is most affected. Examples of this condition associated with central retinal changes are uncommon. A search of the literature reveals reports of a number of cases of typical or atypical retinitis pigmentosa combined with disturbances at the macula. Among the writers who have described such cases are Leber.1Carruthers,2Rieger,3Spare4and Pavia and Dusseldorp,5The ophthalmoscopic picture of a "hole" in the macula in association with retinitis pigmentosa is rare. Leber6in his monumental work on diseases of the retina discussed the presence of macular cysts in association with pigmentary degeneration of the retina and referred to cases reported by Hoffman (1885), Meyer (1889), Noll (1908) and Stock (1908). Leber's article included a photomicrograph of a section of the eye of Stock's