A 79-year-old man was referred to our hospital for detailed examination of right pleural effusion. He was asymptomatic, but physical examination revealed swelling of the right parotid gland. Pleural effusion was exudative with lymphoplasmacytic predominance. A chest computed tomography taken after drainage of pleural effusion showed swelling of mediastinal lymph nodes, a consolidation in the left lower lobe, and a nodule with a low density area in the right lower lobe. Biopsy specimens obtained from a right lower nodule and the right parotid gland showed IgG4-positive lymphoplasmacytic infiltration. Thus IgG4-related disease was diagnosed, and systemic corticosteroid therapy was commenced. Then thoracic lesions and the right parotid gland swelling were resolved, and his corticosteroid dose was tapered. To our knowledge, this appears to be the first case report of IgG4-related disease presenting with pleural effusion and a pulmonary nodule with a low density area.