Abstract: Immunoglobulin G4-related disease (IgG4-RD) is a type of systemic inflammatory diseases that often affect multiple organs. IgG4-related cardiovascular disease (IgG4-RCVD) includes IgG4-related aortic disease, IgG4-related coronary artery disease, IgG4-related pulmonary artery disease, and IgG4-related pericarditis. IgG4-positive plasma cells accumulate and exhibit high expression of glucose transport proteins at the site of IgG4-RCVD involvement. ¹⁸F-fluorodeoxyglucose (FDG) PET/CT can assess the extent and degree of vascular inflammation on a systemic scale by evaluating the metabolic activity of lesions in IgG4-RCVD patients. It plays a pivotal role in diagnosing and quantitatively assessing IgG4-RCVD vascular inflammation, aiding in the selection of biopsy sites, and monitoring treatment efficacy. The authors conducted a comprehensive review of the advancements in the utilization of ¹⁸F-FDG PET/CT for IgG4-RCVD, aiming to furnish clinicians with a valuable reference for diagnosing IgG4-RCVD through the application of ¹⁸F-FDG PET/CT.