BACKGROUND The ocular ischemic syndrome is a rare constellation of ocular signs and symptoms that are secondary to severe carotid artery stenosis. The patient may report decreased vision and ocular pain, while objective findings are variable and include midperipheral dot and blot hemorrhages, rubeosis (with or without neovascular glaucoma), a mid-dilated and poorly reactive pupil, and uveitis. The visual prognosis is generally poor, and treatment is primarily aimed at management of the underlying carotid occlusive disease. METHODS Two cases are presented in which the ocular ischemic syndrome was the initial manifestation of complete bilateral internal carotid artery occlusion. Both patients experienced severe unilateral loss of vision. Internal carotid endarterectomies could not be performed, so medical and surgical management concentrated on maintenance of vascular collateral pathways. CONCLUSIONS The ophthalmic complications of the ocular ischemic syndrome can be devastating--the underlying systemic conditions are life-threatening. It is imperative the clinician be aware of the signs and symptoms of carotid artery disease in order to facilitate prompt diagnosis and appropriate referral.