Abstract: The authors report a case of immunoglobulin G4-related lung disease (IgG4-RLD) with eye and ear damage using ¹⁸F-fluorodeoxyglucose (FDG) PET/CT imaging, accompanied by a review of relevant literature. ¹⁸F-FDG PET/CT imaging of IgG4-RLD with ear damage is clinically rare. Through the analysising of this case, it was demonstrated that ¹⁸F-FDG PET/CT imaging not only accurately assesses the systemic lesion distribution of IgG4-related diseases but also significantly improves the lesion detection rate, providing critical evidence for clinicians to formulate individualized diagnosis and treatment strategies.