Patients with ankylosing spondylitis (AS) may develop cardiovascular manifestations ranging from asymptomatic forms to life threatening conditions. The most important cardiovascular manifestation of AS is aortitis, which frequently involves the aortic root and the ascending aorta leading to valvular insufficiency. The extension of the subaortic fibrotic process into the interventricular septum may cause conduction abnormalities that represent the second common cardiovascular manifestations occurring in AS patients. More rarely, an involvement of coronary arteries and of thoracic and abdominal aorta could be present. Rheumatologists managing AS patients should carefully consider, both in late and in early phases of the disease, the occurrence of an aortic involvement in order to promptly administer adequate treatment.