Abstract: Objective To explore the clinical manifestation, diagnosis, treatment and prognosis of mucosa-associated lymphoid tissue lymphoma of the trachea. Methods Clinical data about one patient with mucosa-associated lymphoid tissue lymphoma of the trachea associated with multiple bacterial colonization were retrospectively analyzed and related literatures were reviewed. Results The patient was a 62 year-old male and initially presented with asthma after activity. Fiberoptic bronchoscope showed a mass in the upper-middle section of trachea. The results of sputum culture showed positive morphological bacteria, Aeromonas salmonicidae, and Enterobacter aerogenes. Then one course of R-CHOP regimen chemotherapy and five courses of R-COP regimen chemotherapy were carried out. The lung mass disappeared after 3 courses of chemotherapy. After 11 months of follow-up, the patient was in good condition, without any signs of recurrence. Conclusion Mucosa-associated lymphoid tissue lymphoma of the trachea is rare, with low risk and good prognosis.