The ocular ischemic syndrome is a progressive disorder that results from chronic hypoperfusion to the eye and orbit, occurring most often from ipsilateral internal carotid or ophthalmic artery occlusion, or severe stenosis. It may also result from other causes of reduced blood flow to the eye and orbit, such as systemic vasculitis. As such, it is associated with a significant risk of both cardiovascular and cerebrovascular disease. The aim of management is to prevent further ocular damage as well as the secondary prevention of systemic co-morbidities. This review discusses the spectrum of clinical presentations in ocular ischemic syndrome, its etiology, and the current status of both ocular and systemic management.