Tracheobronchial amyloidosis is a rare manifestation of the disease and has never been described with FDG PET/CT. In this study, we report a case of a 70-year-old man with increasing dyspnea, a right pleural effusion, a tracheobronchial circumferential wall thickening, and a mediastinal fat infiltration on CT scan. FDG PET/CT revealed intense tracheobronchial uptake associated with mediastinal and intra-abdominal fat uptake. Bronchoscopy and mediastinoscopy with biopsies confirmed the diagnosis of primary amyloidosis and excluded malignancy. FDG PET/CT could be useful for the evaluation of tracheobronchial amyloidosis metabolic activity and follow-up.