Antiphospholipid syndrome (APS) is a rare coagulation disorder associated with recurrent arterial and venous thrombotic events. The hemostatic aspects of antiphospholipid syndrome (APS) in patients requiring thrombectomy along with cardiopulmonary bypass present unique challenges to anesthesiologists. We present the case of a 36-year-old woman with thrombosis of the inferior vena cava as a result of APS with particular reference to the precautions that are necessary during the perioperative care. The particularly important things are the prevention of thrombotic or hemorrhagic complications, management of the associated thrombocytopenia and the laboratory methods of monitoring the perioperative anticoagulation in the setting of a prolonged clotting time.