Ocular ischemic syndrome (OIS) is a group of ocular diseases caused by chronic artery occlusion usually involving the internal carotid artery. Patients suffer from visual loss and pain. OIS is a rare disease which can be confounded with diabetic retinopathy or an older central retinal vein occlusion. The only therapy is to treat the neovascular complications. Due to the high mortality of OIS patients, medical and neurological examinations are mandatory. We discuss the clinical findings and diagnostic and therapeutic options of OIS patients in this paper.