The ocular manifestations of sickle cell hemoglobinopathies, while often not severe enough to affect vision, may develop in proliferative stages, resulting in arteriolar-venular anastomoses, neovascular proliferations, vitreous hemorrhage and retinal detachment. The authors review the historical, biochemical, and geographic aspects of the disease, and provide detailed descriptions of findings in theconjunctiva, uvea and fundus, with particular emphasis on the retina. Pathogenesis, histopathologic and clinical appearance, and modes of treatment are discussed.