Extracranial carotid artery aneurysms (ECAA) are a rare entity, representing 0.34% to 3.7% of peripheral aneuryms, and 0.1% to 2% of all carotid pathology.The natural disease progression carries a 20% major morbidity/mortality rate, mainly from cerebral embolization. Commonly the patient presents complaining of a neck swelling, transient or persistent neurological complaints as hemiparesis and hemiparaesthesia, and other complaints.After aneurysmal imaging intervention is conservative anticoagulation, open surgical aneurysm repair, or endovascular exclusion of the aneurysm from the circulation.Conservative observation is associated with the continuous risk of embolization, as well as the local compression effects. Surgical repair is considered the optimum management line as it lowers the major morbidity/mortality rate to less than 10%, and is the most commonly practiced. Endovascular intervention is still in the experimental phase in ECAAs, however the results of the short-term follow-up are promising.