Immunoglobulin G4-related disease (IgG4-RD) is a chronic immune-mediated disease that may present with tumefactive lesions, fibrosis, and a polyclonal IgG4-positive (IgG4) plasma cell-enriched infiltrate in nearly any anatomic site. IgG4-related disease (IgG4-RD) was described in Japan in the first years of the 21st century, characterized pathologically by the infiltration of IgG4-bearing plasma cells into involved organs.