Here we report on a 61-year-old man with refractory eosinophilic granulomatosis with polyangiitis (EGPA) who presented with dyspnea. Despite treatment with glucocorticoids, intravenous cyclophosphamide, and plasma exchange, his symptoms worsened despite his eosinophil count and myeloperoxidase antineutrophil cytoplasmic antibody titer trending downwards. EGPA with diffuse alveolar hemorrhage was diagnosed on analysis of bronchoalveolar lavage fluid. The patient was treated with rituximab and methylprednisolone pulse therapy and a remission was achieved. He has been receiving mepolizumab since then and remains in remission. It should be recognized that refractory diffuse alveolar hemorrhage can occur in patients with EGPA without elevation of biomarkers if they are receiving systemic corticosteroids.