The ocular ischemic syndrome represents a serious blinding condition due to retinal ischemia secondary to hemodynamically significant carotid artery stenosis. However, this condition has received little attention in the neurological literature and is likely underdiagnosed. We here describe a patient with bilateral severe carotid stenosis and progressive visual loss due to ocular ischemia. The best management for this condition remains controversial and should be established by including these patients into randomized trials of carotid surgery.