A 70-year-old man was referred to our hospital for right pleural effusion. It was exudative and lymphocytic with an elevation of adenosine deaminase (ADA). We suspected tuberculous pleurisy; however, pleural fluid was not diagnosable, so we performed video-assisted thoracic surgical (VATS) pleural biopsy. Histological examination of the right pleura showed numerous IgG4-positive plasma cell infiltrations, and we diagnosed him as IgG4-related respiratory disease. A fluorodeoxyglucose-positron emission tomography (FDG-PET) scan showed an uptake of FDG only in bilateral pleura, but not in other organs. We started corticosteroid therapy, and the volume of bilateral pleural effusion remarkably declined. It is important to consider that IgG4-related disease is one of the differential diagnoses when we see patients with lymphocytic pleural effusion with elevation of ADA in addition to considering the possibilities of tuberculous pleurisy.