Objective: To analyze the clinical and pathologic features and the treatmentoutcomes of primary lymphoma of respiratory system (PLRS). Methods: The clinical manifestation,imaging changes, pathologic subtypes, treatment and overall survival of 11 patients with PLRS wereanalyzed retrospectively. Results: Of the 11 patients diagnosed with PLRS by histopathology, thetumor of 2 patients occurred in trachea and the other 9 in lung. Cough, dyspnea and fever were themost frequent symptoms. Mass or infiltrative changes could be found on the chest X-ray and/or CTscan. Two patients were diagnosed as having Hodgkin's Lymphoma (HL) and 9 having non-Hodgkin'sLymphoma (NHL), including 7 patients with low degree NHL [5 of them (55.6%) were mucosa-associatedlymphoid tissue (MALT) lymphoma] and 2 with intermediate degree NHL. Of 10 patients undergoingexploratory thoracotomy and surgical treatment, 8 received adjuvant chemotherapy and 2 adjuvant ofradiotherapy. The remaining patient was subjected to combined chemotherapy. Both of HL patientssurvived more than 5 years without clinical disease. The median survival of MALT lymphoma and othertype of NHL was 39 months and 34 months respectively. Conclusion: Both the clinical manifestationand imaging changes are non-specific. The diagnosis was made through exploratory thoracotomy (10cases) and fiber-optical bronchoscopy (1 case). MALT lymphoma is the most frequent pathologicsubtype. Majority of patients are diagnosed and treated by surgical resection. The prognosis isacceptable.