The antiphospholipid syndrome (APS) is an autoimmune disorder characterized by the presence of antiphospholipid antibodies (aPL), hypercoagulability leading to vascular thrombosis, and pregnancy morbidity. Since its recognition, a great number of cardiac manifestations have been reported in association with these antibodies, but heart valve abnormalities are the most common cardiac manifestations of the syndrome. They include valve thickness >3 mm, localized thickening involving the leaflet’s proximal or middle portion, and/or irregular nodules on the atrial face of the edge of the mitral valve, and/or the vascular face of the aortic valve. A consensus committee recommended anticoagulation in symptomatic valvular disease. Asymptomatic patients should be prophylactically treated with aspirin.