Described herein is a rarely encountered in clinical practice case regarding surgical management of pathological tortuosity of the aortic arch with a simultaneously present aneurysm thereof. The defect was surgically corrected in the setting of extracorporeal circulation. The tortuous and aneurysmatic aortic arch was resected within the boundaries of the healthy tissues, duly followed by establishing an end-to-end anastomosis between the edges of the resected aorta.