A 72-year-old woman was admitted complaining of productive cough. She had worked for an asbestos factory for twenty years. She was positive for MPO-ANCA. The chest HRCT showed interstitial pneumonia without any UIP pattern. A specimen obtained by video-assisted thoracoscopic lung biopsy revealed chronic interstitial pneumonia associated with an asbestos body. Although the interstitial pneumonia improved after the administration of a corticosteroid and an immunosuppressive agent, hematuria and renal dysfunction developed about nine months later. The serum MPO-ANCA titer was elevated and the renal biopsy specimen revealed the presence of vasculitis. As the interstitial pneumonia improved after this treatment, the correct diagnosis may have been, not asbestosis, but MPO-ANCA-related interstitial pneumonia.