Purpose and background: Bilateral internal carotid artery occlusion is an extremely rare entity, therefore, studies of the natural history of this disease are lacking in the English literature. The purpose of this study is to analyze the natural history and surgical alternatives for patients with bilateral internal carotid artery occlusion. Patient population and methods: Twenty-one patients with bilateral internal carotid artery occlusion were encountered with a mean age of 61 years (range of 48–73 years). Their clinical presentations included eight with hemispheric transient ischemic attacks, three with amaurosis fugax, five with strokes and five with non-hemispheric transient ischemic attack. Diagnosis was confirmed using carotid duplex ultrasound and angiography. The majority of patients had more than one risk factor for atherosclerosis: smoking in 100%. hyperlipidemia in 14/21 (67%), hypertension in 17/21 (81%), coronary artery disease in 15/21 (71%) and diabetes mellitus in 7/21 (33%). In addition to the usual medical treatment, 13 patients underwent surgical intervention: eight had an external carotid endarterectomy, four had a carotid—subclavian bypass (to increase external carotid or vertebral flow for tight stenosis or occlusion of the common carotid or subclavian artery) and one patient had an ascending aorta to innominate artery bypass. At a mean follow-up of 6 years (range 1–11 years), the overall mortality rates were 11/21 (52%). in the surgical group it was 5/13 (38%) and 6/8 (75%) in the medical group. The causes of death included seven myocardial infarctions (four in the surgical group and three in the medical group) and four strokes (one in the surgical group and three in the medical group). In the surgical group, 2/13 (15%) had late neurological deficits (one hemispheric transient ischemic attack and one stroke), in contrast to 6/8 (75%. three strokes and three hemispheric transient ischemic attacks) in the medical group. Conclusions: Bilateral internal carotid artery occlusions have grave prognoses and should be considered a marker for severe systemic disease. Various cerebrovascular reconstructive procedures, if feasible, may be beneficial for some of these patients.