cute retinal necrosis (ARN) is a syndrome characterized by progressive intraretinal inflammation and necrosis. The clinical diagnosis is frequently supported by the identification of the causative infectious agent, most commonly varicella-zoster virus (VZV), though herpes simplex virus (HSV) types 1 and 2, cytomegalovirus, and Epstein-Barr virus (EBV) have also been implicated. 1,2 Epstein-Barr virus, a double-stranded DNA virus transmitted by oral secretions, infects more than 90% of humans. 3 IMPORTANCE Acute retinal necrosis (ARN) is an infectious retinitis primarily caused by the herpesviruses. Although the Epstein-Barr virus (EBV) has been implicated as a cause of ARN, to our knowledge, there has been no histopathologic documentation. We report the clinical history and histopathologic confirmation that EBV can cause ARN.OBSERVATIONS Clinical course and histopathology of a patient diagnosed with ARN caused by infection with EBV confirmed by molecular pathology.CONCLUSIONS AND RELEVANCE Epstein-Barr virus is a recognized cause of intraocular inflammation and has been implicated as a possible cause of ARN. However, to our knowledge, tissue demonstration of EBV in a patient with ARN has not previously been reported. We identified the organism in the necrotic retina of a patient receiving immunosuppression because of idiopathic pulmonary fibrosis.