Antiphospholipid syndrome features not only deep vessel thrombosis but also may have cutaneous manifestations such as Raynaud phenomenon, acral necrosis, livedo reticularis, subcutaneous nodules, and leg ulcers. A 72-year-old man presented with a rapidly progressing leg ulcer. He was already on anticoagulation with warfarin due to atrial fibrillation and disclosed a history of stroke with temporary paresis of the left leg. Histopathology of a biopsy of the edge of the ulcer revealed occlusive arteriosclerosis of medium-sized arteries. Serology showed autoantibodies against cardiolipin, β2- glycoprotein I, and phosphatidylserine which led to the diagnosis of antiphospholipid syndrome. Therapy with low molecular weight heparin, dexamethasone, and azathioprine in combination with stage-adjusted wound care led to complete healing of the ulcer within 5 months.