Forty patients with acquired immunodeficiency syndrome (AIDS) were examined for ocular abnormalities. Twenty of these patients died and the eyes were obtained for culture and histopathologic examination. These patients have multiple opportunistic infections and neoplasms as the result of a severe depression of cellular immunity. Fifty percent of all patients with AIDS and 75% of the autopsy group have ocular signs attributable to AIDS. Ocular findings were confined to four major categories: cytomegalovirus retinitis (10 patients), retinal cotton wool spots (11 patients), conjunctival Kaposi's sarcoma (2 patients) and neuro-ophthalmic motility abnormalities (3 patients). Cytomegalovirus retinitis was a significant cause of visual loss. Seven of 40 autopsy eyes had hand motion or worse visual acuity prior to the patients' death because of CMV retinitis. This necrotic retinitis showed minimal inflammation and progressed to involve the entire retina in three to six months, resulting in a gliotic retinal membrane. Therapy with antiviral agents was not effective. Recognizing the ocular signs of AIDS may facilitate the diagnosis. The ophthalmologist also has a major role in the observation of progression or regression of these ocular manifestations, and can assist in the evaluation of therapy in patients with AIDS.