Immunoglobulin G4 (IgG4)-related disease encompasses a wide variety of immune disorders previously thought be distinct. IgG4-related retroperitoneal fibrosis is one such entity. Metabolic imaging with (18)F-fluorodeoxyglucose ((18)F-FDG) positron emission tomography/computed tomography (PET/CT) can be useful in the management of IgG4-related retroperitoneal fibrosis. We here discuss the case of 63-year-old male with IgG4-related retroperitoneal fibrosis and the role, (18)F-FDG PET/CT played in his management.