The antiphospholipid syndrome is characterized by a combination of laboratory findings (i.e., the presence of at least one antiphospholipid antibody) and clinical manifestations (arterial and/or venous thrombosis, obstetrical complications). Long-term oral anticoagulant is recommended to prevent recurrence of both arterial and venous thrombosis, whereas (low molecular weight) heparin plus aspirin is the treatment of choice to prevent further obstetrical complications. In the rare case of catastrophic antiphospholipid syndrome, heparin plus high-dose corticosteroids plus plasma exchange is associated with the highest recovery rate. Some new, non-antithrombotic-based treatments of antiphospholipid syndrome with rituximab, autologous stem cell transplantation, or hydroxychloroquine are also reviewed.