ObjectiveTo improve the knowledge of primary tracheobronchial lymphoma.MethodsTwo patients with primary tracheobronchial lymphoma admitted to First Affiliated Hospital Guangxi Medical University in 2013 and in 2016 were analyzed retrospectively, and related literatures were reviewed. Chinese National Knowledge Infrastructure, Wanfang database and VIP database were searched by using " trachea lymphoma” or " bronchus lymphoma” as keywords. Meanwhile, databases including PubMed, Ovid Medline and Embase database were retrieved with " Trachea” or " Bronchus” AND " Lymphoma” as keywords. Seventy-two cases of primary tracheobronchial lymphoma were reported, the clinical feature, imaging feature, pathological feature, treatment and prognosis of 72 cases were analyzed.ResultsThe two patients were both females. The chief complaints included cough and dyspnea. The tissue biopsy under bronchoscope was the main diagnostic method. On histopathology, one case was diagnosed grade B cell lymphoma, another was diagnosed mantle cell lymphomas. After chemotherapy, the symptoms were relieved and never relapsed in the follow-up period until October 2016. Literature review found 72 patients with primary tracheobronchial lymphoma with women being the majority (47 cases, 65.28%). The patients aged from16 to 82 years with a median age of 51 years, and nine cases (12.5%) of the patients aged from 16 to 30 years. The specific clinical symptoms were cough and dyspnea. Pulmonary function prompted obstructive dysfunction of pulmonary ventilation. The chest CT scan showed consolidation shadow in trachea and bronchus with or without pulmonary atelectasis. Neoplasms could be found by bronchoscopy. Mucosa-associated lymphoid tissue (MALT) lymphoma was the most common pathological characteristic (36.11%). Mantle cell lymphomas has not been reported currently.ConclusionsPrimary tracheobronchial lymphoma is one of the rare airway tumors. Its clinical features, imaging examinations and bronchoscopic characteristics are not specific, which can be easily confused with lung cancer. It also can cause life-threatening airway obstruction. The bronchoscopy play an important role in diagnosis of primary tracheobronchial lymphoma, while the diagnosis is confirmed by histopathological examination. The main pathology type is MALT lymphoma. The prognosis is good.