Antiphospholipid syndrome (APS) is characterized by a combination of arterial/venous thrombosis or obstetric morbidity and antiphospholipid antibodies such as anticardiolipin antibody or lupus anticoagulant. Digital gangrene is an uncommon manifestation of primary APS. A 61 year-old woman was admitted because of acrocyanosis and progressive digital gangrene in both hands for 1 month. Serologically, lupus anticoagulant was positive. Angiography showed obstruction of digital arteries with collaterals, but there was no evidence of atheromatous plaque or vasculitis. Cardiac echogram did not reveal intracardiac thrombosis or valvular vegetations. She had no evidence of other thrombotic disorders, connective tissue disease, infections or malignancy. Treatment of intravenous heparin and prostaglandin E1 led to stabilization of the symptom, and the necrotic digits were surgically removed. Pathological examination of the amputed digital stump showed arterial and arteriolar thrombotic occlusion, proliferation of capillaries, and ischemic necrosis. After oral anticoagulation therapy, she has been followed without recurrence.We describe the first case of primary APS presenting as digital gangrene in Korea and review the literatures.