Antiphospholipid antibody syndrome has been associated with vascular thrombosis, thrombocytopenia, hemolytic anemia, livedo reticularis, neurologic disorders, and recurrent fetal loss. The diagnosis of antiphospholipid syndrome is given in the presence of an elevated anticardiolipin antibody lupus anticoagulant in addition to a thrombotic event. Antiphospholipid antibodies are responsible for a majority of thrombotic events in children. These antibodies can present as a primary syndrome or secondary to other diseases, such as systemic lupus erythematosus. Anticoagulation therapy with heparin and low-dose aspirin is the recommended treatment in pediatric patients.