The acute retinal necrosis (ARN) syndrome represents a specific pattern of clinical presentation for certain herpes virus infections in the posterior segment of the eye. The classically described triad of the ARN syndrome consists of (1) an arteritis and phlebitis of the retinal and choroidal vasculature, (2) a confluent, necrotizing retinitis that preferentially affects the peripheral retina, and (3) a moderate to severe vitritis. Anterior segment inflammation, optic neuritis, and late retinal detachment are also common features of this disorder. Definitive evidence now implicates at least two members of the herpes virus family; varicella zoster virus and herpes simplex virus as causative agents. This paper summarizes the clinical presentation, as well as the currently recommended treatment regimen for the ARN syndrome, highlighting recent advances that have resulted in a significant improvement in the visual prognosis for affected patients.