Lupus anticoagulant and ACAs are made up of heterogeneous IgG and IgM antibodies that prolong in vitro clotting times and are associated with increased risks of venous and arterial thrombosis, recurrent fetal loss, and autoimmune thrombocytopenia and anemia. These clinical findings with the appropriate laboratory results make up the antiphospholipid antibody syndrome. The antiphospholipid antibodies found in this syndrome are directed against a variety of phospholipid binding proteins of which beta2-glycoprotein and prothrombin are considered to be common antigens. Children who present with thrombosis and are positive for lupus anticoagulant and ACAs have similar clinical presentations and prognoses as adults. Isolated lupus anticoagulant and ACAs in children who are asymptomatic likely do not lead to clinical complications and are transient.