Total occlusion of the contralateral internal carotid artery has often been considered to be a predictor of adverse neurologic outcomes following carotid endarterectomy of an ipsilateral carotid stenosis. Results from both the North American Symptomatic Carotid Endarterectomy Trial and the Asymptomatic Carotid Atherosclerosis Study have suggested this to be true. However, each of these trials had relatively few patients with contralateral occlusion in the surgical arms of the studies. In contrast to these studies, there are multiple surgical series in the literature demonstrating excellent results of carotid endarterectomy in patients with contralateral total occlusion. Recently, advocates of carotid angioplasty and stenting have suggested that this technique may be preferable in patients with a contralateral occlusion because of the perceived poor outcomes with surgery. As carotid angioplasty and stenting becomes more popular, it is becoming even more crucial to better define those patients who are truly at increased risk following carotid endarterectomy; ultimately, this will help clinicians decide which patients may derive the most benefits from endovascular therapies. With these issues in mind, the purpose of this review is to examine results of carotid endarterectomy in patients with total occlusion of the contralateral carotid artery.