The antiphospholipid antibody syndrome (Lupus anticoagulans syndrome) is a rare form of coagulopathy due to the presence of autoantibodies against phospholipids or phospholipid-binding protein cofactors that can lead to vascular thrombosis. We report the case of a 57-year-old female patient presenting with decompensated duodenal stenosis due to a pancreatic tumor. Perioperative testing of coagulation markers revealed with 26 % a strongly decreased Quick-Test and with 81.4 s a prolonged partial thromboplastin time that persisted despite intravenous application of 80 mg vitamin K (Konakion) and 10 units of fresh frozen plasma. Subsequent screening for common causes of thrombophilia revealed antiphospholipid antibodies. Consequently, low molecular weight heparin (Dalteparin-Natrium) was administered perioperatively while a gastroenterostomy with entero-enterostomy was performed with uneventful postoperative course. With this presentation and an analysis of the contemporary literature we would like to discuss different aspects of Lupus anticoagulans syndrome.