Antiphospholipid syndrome (APS) is characterized by the presence of antiphospholipid (aPL) antibodies and clinical features of vascular thrombosis and recurrent fetal loss. The aPL do not target the phospholipids directly but a large number of other molecules among which the principal antigenic target is a phospholipid binding plasma protein- â2 GP-1. APS can exist as primary APS or it may be secondary to other autoimmune conditions, most notably SLE. Hemostatic alterations lead to thrombotic and less commonly, hemorrhagic complications in various organs. The criteria for the diagnosis of APS have been revised in 2006 and comprise clinical evidence of vascular thrombosis or fetal loss and repeated presence of lupus anticoagulant, anticardiolipin antibodies or anti-â2-GP1 antibodies 12 weeks apart. Aspirin and hydroxychloroquine may be protective against development of both venous and arterial thrombosis. APS patients who have had an episode of thrombosis are at a very high-risk of recurrence and require long-term anticoagulation. Anticoagulation is advised during pregnancy for patients who are aPL positive and have a history of repeated pregnancy loss. Corticosteroids, immunosuppressives, IVIG and plasmapheresis may be used for patients with recurrent thrombosis despite anticoagulation. More specifically targeted anti-inflammatory or immunomodulatory therapies and some newer antithrombotics are the potential future treatments for antiphospholipid syndrome.