Acute extracranial carotid artery occlusion constitutes a severe stroke with a potentially catastrophic prognosis and unfavorable natural history. In accordance with established stroke treatment protocols and penumbral imaging findings, further intervention is indicated following unsuccessful conservative or thrombolytic approaches. Although preferred today in intracranial circulation, the endovascular approach has failed to achieve similar satisfactory results in extracranial carotid artery occlusion. Additional to interventional radiology, carotid endarterectomy offers a viable alternative that can achieve recanalization promptly and yield comparable clinical outcomes. The chapter briefly discusses the natural history, pathophysiology, diagnostic prospects and treatment options in patients with extracranial carotid artery occlusion, with a focus on surgical intervention.