The classification of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) has limitations because the condition includes disorders with similar general clinical features, similar characteristics of lung and renal involvement and a positive ANCA serology. A 40-year-old woman was admitted to our hospital for hemoptysis and dyspnea. She had no history of bronchial asthma. Laboratory examinations revealed hypereosinophilia, positive anti-myeloperoxidase antibodies, hematuria and proteinuria. The patient was ultimately diagnosed with AAV associated with diffuse alveolar hemorrhage, rapidly progressive glomerulonephritis and hypereosinophilia without bronchial asthma. Obtaining a definitive diagnosis of ANCA vasculitis can be very difficult, and the characteristics of this case were not compatible with the findings of typical AVV. We herein report a rare case of AVV.