Abstract: The authors reported a case of Sjögren′s syndrome (SS) with lung involvement, detailed the clinical symptoms, CT and ¹⁸F-fluorodexyglucose (FDG) PET/CT features of the disease, and deepened the understanding of SS through reviewing the literature. The main findings of ¹⁸F-FDG PET/CT imaging in patients with SS lung involvement were multiple nodules and patches in both lungs, and pulmonary cyst-like lesions, and some lesions were accompanied by mild uptake of ¹⁸F-FDG. ¹⁸F-FDG PET/CT imaging can be used to evaluate multi-system lesions and disease activity of SS, and help distinguish it from neoplastic lesions.