Aortic involvement is a potential life-threatening complication of ankylosing spondylitis, usually occurring late in the course of this frequent disease. Inflammatory lesions evolving to fibrosis are primarily localized in the aortic root causing regurgitation, but this process can extend into the left atrium (subaortic bump) involving the mitral valve and the heart conduction system. First, second and third degree atrioventricular blocks are the most common conduction alterations described and they can be temporary. Chronic periaortitis has been described in ankylosing spondylitis patients. This disease is characterized by inflammation evolving to fibrosis and it is localized in the periaortic and peri-iliac retroperitoneum. It causes compressive effects on ureters and venous, arterial and lymphatic vessels. Its treatment employs endoscopic and/or surgical procedures and administration of corticosteroids, even in association with immunosuppressive agents. Both aortitis (with conduction system alterations) and periaortitis should be kept in mind by the physicians because they can significantly influence the prognosis of ankylosing spondylitis patients and they can need a rapid treatment.