Retinal artery occlusion leads to profound visual impairment in the affected eye. It is rarely caused by local ophthalmic pathologies. Most patients present with a large number of cardio- and cerebrovascular risk factors. Visual loss is the leading symptom in central retinal artery occlusion (CRAO), whereas a circumscribed visual field defect is claimed in branch retinal artery occlusion (BRAO). Although many attempts have been made to improve the course of the disease, no effective therapy is available. There is some hope that intravenous fibrinolysis could influence the natural course but the therapeutic window is small (ca. 4.5 h), and treatment efficacy is still not proven. It is important for ophthalmologists to guide the patients to a comprehensive and prompt neurological and cardiological diagnostic work-up.