Antiphospholipid syndrome (APS) is characterized by recurrent venous or arterial thromboses, fetal losses and thrombocytopenia in the presence of antiphospholipid antibodies, namely lupus anticoagulant, anticardiolipin antibodies or antibodies directed to various proteins, mainly β2 glycoprotein I, or all three. There is consensus in treating patients with APS and first venous thrombosis with oral anticoagulation to a target international normalized ratio (INR) of 2.0–3.0. A recent systematic review recommended a target INR of >3.0 in those patients with APS and arterial thrombosis. The approach in women with obstetric manifestations of APS is based on the use of aspirin plus heparin. The best treatment for patients with the catastrophic variant of the APS is a combination of anticoagulation, corticosteroids, and plasma exchange or intravenous immunoglobulins.