Anti-glomerular basement membrane disease (anti-GBM disease) associated with renal and lung lesions has a poor prognosis. Diffuse alveolar hemorrhage (DAH) is a complication that worsens anti-GBM disease prognosis. We report a rescue case using veno-venous extracorporeal membrane oxygenation (VV-ECMO) for diffuse alveolar hemorrhage due to isolated pulmonary anti-GBM disease; a rare anti-GBM syndrome. A 30-year-old Japanese female with no past medical history. Presented with acute hypoxemic respiratory failure requiring mechanical ventilation. Progressive deterioration and refractory hypoxemia prompted therapy with VV-ECMO. Serum anti-GBM antibody confirmed the diagnosis of anti-GBM disease. Multi-modal systemic therapy with pulse-dosed methylprednisolone, plasma exchange, and rituximab resulted in significant clinical improvement. VV-ECMO for 10 days was uncomplicated. Renal replacement therapy was not required. The patient was extubated on day 18 and discharged from the hospital after 45 days. VV-ECMO supportive therapy for DAH with refractory respiratory failure was demonstrated to be effective pending definitive diagnostic and therapeutic management in this case of isolated pulmonary anti-GBM disease.