We present here two patients whose near fatal respiratory distress was caused by pulmonary hemorrhage, and who were treated successfully by extracorporeal membrane oxygenation (ECMO). The underlying disease was anti-neutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitis. They were initially treated with methylprednisolone pulse therapy along with cyclophosphamide. However, their respiratory failure progressed with a low PaO2/FiO2 ratio (< 100 mmHg) despite mechanical ventilation, and ECMO was initiated. After several days, the pulmonary hemorrhage subsided, and the patients were weaned successfully from ECMO. We suggest that ECMO may be used to manage life-threatening pulmonary hemorrhage in patients suffering from ANCA-associated systemic vasculitis.