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Atherosclerotic occlusive disease of the craniocervical arteries has particular importance within the overall spectrum of cerebrovascular disease, particularly as a predictor for the presence of systemic atherosclerosis. Patients with symptomatic stenosis or occlusion of these arteries, or those with ''large-artery'' ischemic stroke (IS), have specific clinical features. They may have (1) a risk factor profile that differs from other IS subtypes, (2) an increased association with cardiac and other systemic atherosclerosis, and (3) an increased risk of early stroke recurrence, and they may benefit from revascularization therapies in addition to medical management strategies.