• Patients with acute ischemic stroke due to atherosclerotic carotid artery occlusion (ACAO) should receive intravenous tissue plasminogen activator (alteplase) if they meet eligibility criteria of the National Institute of Neurological Disorders and Stroke (NINDS) or the European Cooperative Acute Stroke Study III (ECASS III). • Patients with acute stroke due to ACAO who are not eligible for intravenous tissue plasminogen activator should receive aspirin. Heparin or heparin-like drugs do not improve outcome and should not be used. • Therapy for prevention of recurrent stroke in patients with ACAO should consist of lifestyle modifications, risk factor intervention, and antiplatelet drugs. Warfarin is not indicated. • Extracranial-intracranial bypass surgery provides no benefit over medical therapy in preventing recurrent stroke in a general population of patients with ACAO or in any subgroups selected by clinical, arteriographic, or hemodynamic criteria. • Other surgical or endovascular procedures have no proven value in treating or preventing stroke due to ACAO. • Asymptomatic carotid occlusion has a benign prognosis and requires no specific treatment other than lifestyle modification and risk factor intervention.