Kinking and coiling of the internal carotid artery (ICA) sometimes may result in symptomatic cerebrovascular disease, but indisputable evidence linking the two conditions is lacking. However, there is enough evidence to warrant careful consideration of surgical correction in patients who have features of the carotid artery syndrome and kinking of the ICA as shown on angiography. Kinking or buckling of the artery is due to atherosclerosis and is to be distinguished from coiling, which is ascribed to embryological causes. Definite recommendations regarding the advisability of surgery for infants who are discovered to have coils cannot be made, but coiling is generally asymptomatic. Adults with kinks in their carotid arteries who have recurrent transient ischemic attacks (TIAs) benefit most from surgical correction, particularly if symptoms are aggravated on head rotation, which may cause the kink to obstruct.