A 46-year-old man with a 4-month history of bronchial asthma was admitted to our hospital complaining of progressive dyspnea, weakness of the lower extremities, multiple truncal erythematous purpura and hemoptysis. Neurological examination identified the presence of mononeuritis multiplex. Laboratory data indicated marked anemia, eosinophilia, severe renal failure with nephrotic condition and elevated serum myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA) levels (1,050 EU). Chest computed tomography showed diffuse ground glass opacity in both lungs. Bronchoalveolar lavage fluid revealed bloody fluid with eosinophilia (81%). Microscopic findings of a transbronchial lung biopsy were consistent with alveolar hemorrhage. A skin biopsy revealed eosinophilic vasculitis consistent with Churg-Strauss syndrome (CSS). A renal biopsy specimen revealed pauci-immune crescentic necrotizing glomerulonephritis. Consequently, he was diagnosed as having CSS presenting with diffuse alveolar hemorrhage (DAH) and rapidly progressive glomerulonephritis (RPGN) with MPO-ANCA-associated systemic vasculitis. His clinical condition markedly improved with the administration of intravenous corticosteroid (CS) and cyclophosphamide (CY). Thus, we report a case of CSS presenting with the rare complication of DAH and RPGN.