To report a case with primary lung mucosa-associated lymphoid tissue type marginal zone B-cell lymphoma (PMZL-MALT). The patient was admitted in our hospital mainly due to intermittent cough and expectoration for 4 years, and aggravating for 1 year. The anti-infective treatment was given to this patient, and the efficacy was not good. Laboratory tests showed no specific indicators. Chest enhancement CT examination showed pulmonary consolidation lesions. After taking biopsy by tracheoscopy and pathological examination, the diagnosis of mucosa-associated lymphoid tissue type marginal zone B-cell lymphoma was confirmed. After receiving chemotherapy, the patient remains under treatment in a stable condition and regular reexamination.