We studied the clinical and histopathologic characteristics of the eyes obtained after death from a patient with adult-onset foveomacular pigment epithelial dystrophy. The pigmentation seen in the central fovea corresponded histologically to a hyperplastic clump of retinal pigment epithelium. The pale yellow rim surrounding the central pigmentation corresponded histologically to dense periodic acid-Schiff-positive material underlying thinned, atrophic retinal pigment epithelium. Fluorescence microscopy demonstrated homogeneous autofluorescence in the retinal pigment epithelium that was similar in intensity to that of an age-matched control. The results of this clinicopathologic study suggest that in adult-onset foveomacular pigment epithelial dystrophy, an alteration of macular retinal pigment epithelium causes an accumulation of abnormal subretinal pigment epithelial material, photoreceptor degeneration, and serous retinal detachment.