Six patients had macular vitelliform lesions similar to those in Best's vitelliform foveal dystrophy but all had normal electro-oculograms (EOG) and no familial involvement. Two patients had an exudative form of degenerative chroidopathy. The remining four had vitelliform lesions of unknown etiology. Fluorescein angiography demonstrated slight hyperfluorescence through rarefied retinal pigment epithelium but no leakage occurred from perifoveal retinal capillaries. The diagnosis of vitelliform foveal dystrophy should be restricted to those patients who have the morphologic lesions, and abnormal EOG, and a contributory family history.