ObjectiveTo discuss the clinical features, diagnosis, treatment and prognosis of primary tracheobronchial amyloidosis. MethodsWe retrospectively studied 50 primary tracheobronchial amyloidosis cases reported in Chinese literature during 1989-2012 and one in our hospital. The clinical information, symptoms, imaging findings, endoscopic manifestations and treatment were analyzed. ResultsThe patient in our hospital was admitted due to “short breath”. Chest CT , bronchoscopy and pulmonary function examination were done; bronchoscopy biopsy specimen confirmed the diagnosis of PTA; and the patients responded to cyclophosphamide treatment. The main clinical symptoms of the 50 patients included cough (96%, 48/50), short breath (70%,35/50), sputum (66%,33/50), fever (24%,12/50), and hemoptysis (22%,11/50). Thickening of lung markings (45.5%,15/33)was the most common chest X-ray finding; airway wall thickening and stenosis (76.2%,32/42) was the most common CT manifestation; and the most common endoscopic finding was also airway wall thickening and stenosis (40.8%,20/49), followed by mucosal congestion and edema (32.7%,16/49). Obstructive ventilatory dysfunction was found to be the major problem during pulmonary function tests. The diagnoses in 47(94%) patients were confirmed by bronchoscopic biopsy. Twenty-one literatures reported complications in 3 cases and misdiagnosis in 24 cases. Forty-two of the 50 patients received drug treatment, bronchoscopic interventional therapy, external beam radiation therapy and surgical treatment, and they responded well.ConclusionPrimary tracheobronchial amyloidosis is rare and is prone to be misdiagnosed. Correct diagnose needs pathological examination and currently there is no specific treatment for it.