Diffuse alveolar hemorrhage (DAH) is an uncommon complication in patients with systemic lupus erythematosus (SLE), and mortality remains high. In recent years, cases of DAH due to SLE treated with extracorporeal membrane oxygenation (ECMO) have rarely been reported. The authors present a case of a 43-year-old woman with SLE who had rapidly aggravating dyspnea and hemoptysis. She was diagnosed as having DAH with refractory respiratory failure and was successfully managed with veno-venous ECMO. We propose ECMO as a useful salvage therapy in patients with alveolar hemorrhage secondary to SLE who are failing conventional ventilatory support.