Abstract: IgG4-related disease(IgG4-RD) is a chronic autoimmune disease implicating multiple organs and tissues. Its pathological characteristics are infiltration of lymphocytes and IgG4-positive plasma cells in the implicated organs. Pulmonary involvement reportedly varies. Here we reported a case of IgG4-RD with the onset of cough and dyspnea, presenting with multiple lesions. The pulmonary involvement was manifested as diffuse nodules in lungs that dissolved after glucocorticoid therapy.