The antiphospholipid antibody syndrome (APLAS), though an uncommon entity involves multiple organs in the body. The antiphospholipid antibodies (APLA) refer to several groups of autoantibodies against negatively charged phospholipids occurring independently or in association with systemic lupus erythematosus (SLE) and related autoimmune disorders. Several studies to date found those patients with APLA, predominantly IgG and to lesser extent IgM isotype and lupus anticoagulant (LAC) are associated with arterial and venous thrombosis, recurrent fetal loss, thrombocytopenia, and livedo reticularis. We have described two cases of APLAS, one primary and the other secondary, their management and cardiac manifestations. Cardiac manifestations of the syndrome include coronary artery thrombosis and valvular heart disease. These can be serious and difficult to treat. Although the exact treatment of the cardiac manifestations of APLAS is not clear, anticoagulation is the currently recommended therapy.