We present a case of central retinal artery occlusion (CRAO) in a 59-year-old male suffering from infective endocarditis. The patient was receiving antibiotic treatment for the endocarditis for 5 days prior to the event and received conservative treatment for the CRAO without any improvement. A few days after the event, the patient underwent heart surgery with aortic and mitral valve replacement and eventually recovered without any other side-effects. CRAO is a rare but devastating embolic complication of infective endocarditis and can happen regardless of whether a patient is receiving systemic antibiotic treatment or not. These patients need close observation, because an immediate diagnosis and an urgent therapeutic procedure are mandatory, as the prognosis for vision is poor.