ABSTRACT: Patients with AIDS have an abnormality of retina/optic nerve function, manifested as decreased contrast sensitivity (in the absence of ocular opportunistic infections or media opacity), abnormalities on automated perimetry, and loss of retinal nerve fiber layer, even among those with good visual acuity, termed the "human immunodeficiency virus (HIV) neuroretinal disorder." The objectives of this study were to determine the prevalence, incidence, risk factors, and outcomes of HIV neuroretinal disorder.Prospective cohort study.A total of 1822 patients with AIDS without ocular infections or media opacities.Patients with HIV neuroretinal disorder were identified by a contrast sensitivity <1.50 log units in either eye in the absence of ocular opportunistic infections or media opacity.Incidence of HIV neuroretinal disorder, mortality, visual impairment (visual acuity ?20/50), and blindness (?20/200) on logarithmic visual acuity charts.Sixteen percent of participants had HIV neuroretinal disorder at enrollment. The estimated cumulative incidence by 20 years after AIDS diagnosis was 51% (95% confidence interval [CI], 46-55). Human immunodeficiency virus neuroretinal disorder was more common in women and African Americans. Risk factors for HIV neuroretinal disorder included hepatitis C infection, low CD4+ T cells, and detectable HIV RNA in the blood. Patients with HIV neuroretinal disorder had a 70% excess mortality versus those without it, even after adjusting for CD4+ T cells and HIV load (hazard ratio [HR], 1.7; 95% CI, 1.3-2.1; P < 0.0001). Patients with HIV neuroretinal disorder had increased risks of bilateral visual impairment (HR, 6.5; 95% CI, 2.6-10.6; P < 0.0001) and blindness (HR, 5.9; 95% CI, 2.8-13.7; P = 0.01) versus those without HIV neuroretinal disorder.Human immunodeficiency virus neuroretinal disorder is a common finding among patients with AIDS, and it is associated with an increased mortality and an increased risk of visual impairment. Successful antiretroviral therapy decreases but does not eliminate the risk of HIV neuroretinal disorder.