Abstract Extracranial internal carotid artery aneurysms (EICAA) are uncommon lesion. These aneurysms can be classified as true or false aneurysms, atherosclerotic, dysplastic, infectious, posttraumatic and iatrogenic aneurysms. The most common presentation is central neurologic dysfunction, either a stroke or a transient ischemic attack. The rupture of these aneurysms can lead to severely impairment and can affect the quality of life of the patients or even may lead to death. Management of these lesions is required in most cases to prevent complications, however there is no treatment guideline or expert consensus for the management. We present a case of an unusual EICAA, associated with kinking of the affected vessel and review the literature.