Abstract: The authors report a case of immunoglobulin G4 (IgG4)-related lung disease with eye and ear damage using PET/CT imaging, accompanied by a review of relevant literature. . PET/CT imaging of IgG4-related lung disease with ear damage is clinically rare. Through the analysising of this case, it was demonstrated that 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.