Extracranial carotid artery aneurysm (ECAA) is an uncommon but serious condition. The morbidity and mortality of ECAA are assumed to be high when untreated. ECAA treatment presents a challenge because of accessibility of the carotid artery and lack of evidence-based guidelines. When exclusion of the aneurysm is considered, surgical resection of the aneurysm with reconstruction of blood flow is still considered the gold standard. Several alternative and endovascular approaches are discussed.