We reviewed the clinical records of 47 patients (92 eyes) at the Wilmer Institute in whom foveomacular dystrophy had been diagnosed between 1977 and 1990 to determine reasons for initial examination, long-term visual prognosis, and the factors associated with visual loss. From each clinical record, we obtained the patient's age at the time first seen, symptoms, initial and final visual acuity, lesion size and appearance, and results of fluorescein angiography, electroretinography, and electro-oculography. The most common reasons for examination were decreased visual acuity in 25 of the 47 patients (53%), metamorphopsia in six (13%), referral for diagnosis in seven (15%), and misdiagnosis of age-related macular degeneration in seven (15%). Over an average follow-up period of 5.0 years, in 29 patients (56 eyes), visual acuity changed less than two lines in 41 eyes and decreased greater than or equal to two lines in 15 eyes. In most patients with foveomacular dystrophy, visual acuity appears to remain relatively stable.