Ocular manifestations can occur in up to 50% of human immunodeficiency virus (HIV)/acquired immune deficiency syndrome (AIDS) patients and posterior segment involvement is the most common presentation. The posterior segment manifestations of AIDS can be divided into four categories: retinal vasculopathy, opportunistic infections, unusual malignancies and neuro-ophthalmologic abnormalities. Retinal microvasculopathy and cytomegalovirus (CMV) retinitis are the most common manifestations, even in the era of highly active anti-retroviral therapy (HAART). Highly active anti-retroviral therapy has been shown to cause regression of CMV retinitis, reduce the incidence of CMV-related retinal detachments, and prolong patient survival. Immune recovery uveitis is a new cause of vision loss in patients on HAART. Diagnosis and treatment are guided by the particular conditions and immune status of the patient.