Immunoglobulin G4-related disease (IgG4-RD) is a rare immune-mediated fibroinflammatory condition that tends to form tumor-like lesions in multiple organs. Patients usually present with serum IgG4 elevation and various imaging manifestations. The histopathological hallmarks of IgG4-RD are dense lymphoplasmacytic infiltration, storiform fibrosis, and obliterative phlebitis. We present the case of a 42-year-old man with dry eye and bilateral submandibular swelling; IgG4-RD was diagnosed after serial examinations.