The antiphospholipid syndrome is characterized by the presence of arterial or venous thrombosis or recurrent miscarriages in a patient with positive laboratory tests for antiphospholipid antibodies (anticardiolipin antibodies and/or lupus anticoagulant and/or anti-beta2-glycoprotein I). Despite the strong association between antiphospholipid antibodies and thrombosis and obstetric morbidity, their pathogenic role in the development of these clinical features has not been fully elucidated. With respect to the treatment, at present there is consensus in treating patients with thrombosis with longterm oral anticoagulation. The approach for women with obstetric manifestations is based on the use of aspirin and heparin. However, the knowledge of new pathogenic mechanisms might identify novel therapeutic targets and therefore may improve the management of these patients.