Antiphospholipid syndrome is a diagnosis with the clinical manifestations of thromboses in the presence of an antiphospholipid antibody. A 25-year-old man with a history of deep venous thrombosis, pulmonary emboli, and myocardial infarction, and receiving long-term anticoagulation with warfarin, all due to primary antiphospholipid syndrome, presented with blue toe syndrome from a primary superficial femoral artery thrombus. He was anticoagulated with fondaparinux in addition to dipyridamole and aspirin perioperatively. The area of thrombus was resected and reconstructed using a cephalic vein interposition graft. This report reviews antiphospholipid syndrome and identifies potential questions and problems relating to a rare clinical presentation.