Objective To improve the knowledge of pulmonary mucosa-associated lymphoid tissue (MALT)lymphoma. Methods A patient diagnosed as pulmonary MALT lymphoma was reported and related literatures were reviewed. Results The patient was a 58-year-old male,admitted due to intermittent fever,cough,sputum production,chest tightness and fatigue for 4 years.The patient was diagnosed as "pulmonary tuberculosis and tuberculous pleurisy" in other hospital and received anti-tuberculosis treatment for 3 years.The CT of the chest showed consolidation in the right middle lobe,right low lobe and left lower lobe with bronchial ventilation levy,miliary nodules in the right middle lobe,interstitial thickening,and right pleural effusion.Ultrasound guided lung biopsy pathology of the right lung showed diffuse small lymphocytes infiltration.The immunohistochemistry showed positive staining of CD20,CD79α and Vim,and weakly positive staining of Ki67(15%).Therefore,the patient was finally diagnosed pulmonary MALT lymphoma. Conclusions Pulmonary MALT lymphoma has no specific clinical manifestations,so is easy to be misdiagnosed as pulmonary tuberculosis,pneumonia or lung cancer.The patients with suspicious pulmonary MALT lymphoma should undergo percutaneous lung biopsy,transbronchial lung biopsy or open lung biopsy as soon as possible for immunohistochemistry staining to confirm the diagnosis.