The significance and management of cervical carotid artery tortuosity are controversial. One hundred eighteen cases of angulation of the common carotid artery and over 800 cases of elongation of the internal carotid artery have been reported. The etiology, presentation, diagnosis, and treatment of each entity are reviewed. Angulation of the common carotid artery is often managed conservatively. For clarity, elongations of the internal carotid artery should be classified as either tortuosities or kinks. Some patients with kinks of the internal carotid artery will have episodes of cerebrovascular insufficiency related to the position of their heads. Such patients merit thorough diagnostic evaluation. When other etiologic factors have been eliminated, patients in this group may benefit from a surgical procedure directed at the vascular abnormality. Applicable surgical techniques are discussed.