The acquired immune deficiency syndrome (AIDS) is a recently described disorder of cellular immunity in homosexuals, intravenous drug abusers, and Haitians. Manifestations include Kaposi's sarcoma, Pneumocystis carinii pneumonia, and other opportunistic infections. Ophthalmic and autopsy examinations of 30 patients at UCLA revealed frequent ocular abnormalities. Findings included cotton-wool spots (16 patients), cytomegalovirus retinitis (eight patients), conjunctivitis and keratitis (four patients), conjunctival Kaposi's sarcoma (three patients), Mycobacterium avium intracellulare choroidal granulomas (one patient), and retinal periphlebitis (one patient). We feel that the presence of cotton-wool spots in patients with this syndrome indicates a poor prognosis. Immunologic and electronmicroscopic studies of cotton-wool spots revealed no infectious agents or immunoglobulin deposition. Cytomegalovirus retinitis always was associated with a fatal outcome. The retinitis was characterized by an acute inflammatory reaction in 50% of patients. Ophthalmologists should be aware of the syndrome and its ocular manifestations.