A 69-year-old male was referred to our hospital due to a history of dry cough and abnormalities on chest X-ray. Three months after the initial visit, chest computed tomography revealed pleural effusion and mediastinal lymph node enlargement, in addition to bilateral subpleural reticulation. Serum IgG4 was elevated (475 mg/dL). Lung biopsy specimens obtained by video-assisted surgery revealed marked lymphoplasmacytic infiltration in the pleura, interlobular and peribronchial interstitium, and alveolar walls, and obliterative phlebitis and arteritis accompanied by intimal and mural inflammatory cell infiltration. In addition, immunohistochemical staining of IgG4 showed numerous IgG4-positive plasma cells. Consequently, he was diagnosed with IgG4-related respiratory disease and treated with prednisolone (30 mg/day). As a result, his clinical condition and chest imaging abnormalities markedly improved.