The syndrome characterized by papillomacular bundle damage, central or cecocentral scotoma, and reduction of color vision can be produced by toxic, nutritional, or hereditary causes. Patients who present with such a picture should be evaluated for toxic sources such as tobacco or alcohol use. Nutritional deficiencies must be considered and a good family history should be obtained to explore hereditary factors. DNA mutation analysis is available to detect genetic abnormalities. A trial of nutritional replacement may be advisable even in the face of normal laboratory values. Previous reports have shown dramatic visual improvement with intramuscular hydroxocobalamin and other oral vitamin B complexes, even when specific deficiencies are not found.