Recent several reports showed that 18F-fluorodeoxyglucose-positron emission tomography/computed tomography (FDG-PET/CT) scans accurately, and safely identifies the multiple organs' involvements in IgG4-related diseases (IgG4-RD) patients. Disease activity in two patients, aged 74 and 73 years (serum IgG4, 807 and 1630 mg/dl, respectively), with IgG4-RD were monitored using FDG-PET/CT. Scans before treatment showed splenomegaly with high FDG uptake. Inflammatory aneurysm, submandibular gland swelling, splenomegaly and tubulointerstitial nephritis resolved after treatment with prednisolone. Follow-up FDG-PET/CT at 4 months, with no symptoms, showed a significant decrease in FDG accumulation and improvement of splenomegaly in splenic lesion of IgG4-RD. Thus, splenic lesions detected by FDG-PET/CT might be novel involvements of IgG4-RD.