The study focuses on modern approaches to diagnostics and surgical treatment of patients with aneurysms of the thoracic aorta combined with congenital pathologies of the aortic arch and brachiocephalic arteries. The effect of hypothermia, extracorporeal blood circulation, cerebral perfusion, preventive transposition of the aberrant subclavian arteries is evaluated in the context of prevention of postoperative complications. 34 patients with aneurysms of the thoracic aorta were included in the study. Aortic aneurysms were found in 22 of patients with congenital aortic arch defects and in 12 patients with an abnormal position of the arch and/or brachiocephalic arteries. Aberrant origin of the subclavian arteries is the most common defect of the arch in the case of thoracic aorta aneurysms. Aortic arch kinking and congenital anomalies of the aortic arch significantly influence the tactic of surgical treatment of aneurysms because of a high probability of clamping the aortic arch branches and neurological complications. Therefore, prevention of cerebral complications is the primary objective of surgery. Cerebral perfusion or staged surgical interventions also find extensive use. A congenital aortic arch in a number of patients determines a surgical approach and influences the choice of techniques. Surgical treatment of aortic aneurysms associated with congenital anomalies or kinking of the aortic arch depends on a set of several factors. The surgical decision must be based on precise preoperative diagnosis and detailed analysis of all individual anatomical characteristics of the aortic arch and its branches. The safety and efficacy of operations can only be achieved on the basis of a differentiated and individual approach.Received 6 October 2016. Accepted 11 December 2016.Funding: The study had no sponsorship.Conflict of interest: The authors declare no conflict of interest.Author contributionsAll authors meet ICMJE authorship criteria and contributed equally at all stages of the research.