Extracranial internal carotid artery atherosclerotic occlusive disease is a common ischemic stroke mechanism. Vascular risk factor control remains the cornerstone of stroke prevention in patients with both asymptomatic and symptomatic carotid occlusive diseases. Intensive medical therapy refers to the contemporary approach of antiplatelet therapy, blood pressure control, low-density lipoprotein reduction, and lifestyle modification to reduce stroke risk. Carotid revascularization with endarterectomy or angioplasty and stenting are established treatments for patients with symptomatic carotid stenosis ≥70%. Previously accepted ischemic stroke preventative strategies, such as carotid revascularization for asymptomatic carotid stenosis, require reassessment given advances in both medical therapy and surgical techniques. The purpose of this review is to describe contemporary approaches to the management of extracranial carotid atherosclerotic occlusive disease and the basis of these recommendations. Results from recently published clinical trials will be highlighted in addition to updated information from clinical trials addressing knowledge gaps in prevention of stroke caused by extracranial disease.