In 1909, Dr. Stargardt described seven patients who developed a juvenile onset macular degeneration with a striking presentation of yellowish flecks surrounding the macula. The eponym "Stargardt's disease" has since been used to describe this condition. However, there is some ambiguity in the literature concerning the characteristics which differentiate this condition from other hereditary retinal dystrophies. We have undertaken a review of the literature in an attempt to clarify the parameters of Stargardt's disease.