Lipofuscin is an intracellular aging pigment with fluorescent properties, found in retinal pigment epithelium cells of the eye. It is the main fluorophore used in fundus autofluorescence imaging techniques to diagnose, describe, and follow retinal disease. Lipofuscin forms by incomplete lysosomal degradation of cellular material previously subjected to oxidative changes. A2E is the most studied fluorescent component of lipofuscin, but most of its composition remains unknown. Lipofuscin is photoreactive, generating reactive oxygen species, which may explain its role in disease development. Further knowledge is needed concerning lipofuscin genesis, biochemical composition, fluorescent compounds, and role in pathogenesis of retinal degenerative disease.