Acute macular neuroretinopathy (AMNR) is a rare condition that produces transient or permanent visual impairment. Typical cases have acute onset multifocal scotomas that correspond rather precisely with reddish, flat, or depressed circumscribed lesions in the macula. These lesions are wedge-shaped and generally point toward the fovea. The pathophysiology of AMNR is unclear, the causes are uncertain, and there is no specific treatment for this condition. This review summarizes the presentation, possible risk factors, and prognosis of the 41 cases of AMNR reported in the published, English-language literature from 1975 through April 2002. Possible areas for future research into the etiology of this rare condition are discussed.