The acute retinal necrosis syndrome is characterized by necrotizing retinitis, vitritis, and retinal vasculitis occurring in otherwise healthy patients. Experience with 11 cases and the review of 30 additional cases in the literature are presented. In this series, 50% of the affected eyes developed retinal detachments, and 64% had a final visual acuity of less than 20/200. The natural history, diagnosis, postulated etiology, and suggestions for management will be discussed.