True vitelliform dystrophy rarely appears in the adult population. We describe 10 cases in adults of bilateral vitelliform lesions clinically mistaken for Best's disease. Fluorescein angiography is a useful tool in distinguishing this dystrophy from Best's disease or other diseases. The angiographic findings suggest pigment epithelial disease. Adult vitelliform degeneration may lead to dry atrophic macular degeneration in a similar fashion as macular drusen. Symptoms and visual findings in these patients are fairly stable, and may be only slowly progressive in spite of ophthalmoscopic and fluorescein angiographic changes over a period of years. The electro-oculogram is useful in separating adult vitelliform macular degeneration from true vitelliform dystrophy.