The literature on the etiology, pathogenesis, clinical manifestations and pathology, basic approaches to the treatment of congenital and acquired pathological deformation of the internal carotid artery has been analyzed. The review discusses the possible risk factors and diseases that lead to the development of pathological deformations as well as existing hypotheses of pathogenesis. Open and unresolved issues of the etiology and pathogenesis of this disease are identified. The disputable issues on the emergence and development of vascular deformations in children, young people and elderly are discussed. The authors posit a hypothesis that congenital and acquired pathological deformations are different diseases which differ by etiology, pathogenesis, clinical and pathomorphological picture, prognosis, approaches to diagnosis and treatment; the relationship between them has not been proved.