PURPOSE: To determine the cause of bilateral retrobulbar optic neuritis followed by progressive outer retinal necrosis in a patient with human immunodeficiency virus (HIV). METHODS: Extensive ophthalmologic, neurologic, infectious disease, rheumatologic, and radiologic examinations were performed. RESULTS: Cerebrospinal fluid samples taken after the onset of bilateral retrobulbar optic neuritis and before the development of clinical progressive outer retinal necrosis disclosed varicella zoster virus from polymerase chain reaction and viral culture. CONCLUSION: Ophthalmologists and neurologists should consider varicella-zoster virus optic neuritis as a potential precursor of progressive outer retinal necrosis and as a cause of retrobulbar optic neuritis in patients infected with HIV.