Pulmonary B-cell lymphoma in the extranodal marginal zone of mucosa-associated lymphoid tissue (MALT), a rare tumor originating from bronchial mucosa-associated lymphoid tissue, is the major histologic type of primary pulmonary lymphoma. Combined lung squamous cell carcinoma with pulmonary MALT lymphoma is rare. A 63-year-old male patient presented to the hospital because of a dry cough, and chest CT showed soft tissue density nodules in the upper lobe of the right lung, the boundary was visible lobulation and spiculation, and the middle lobe of the right lung showed patchy shadow, moderate enhancement, associated with bronchial traction. After a multidisciplinary diagnosis and treatment (MDT) discussion, surgical resection was done for the patient, and postoperative pathological results showed pulmonary MALT lymphoma combined with lung squamous carcinoma. For complex pulmonary multiple lesions, judgment needs to be made after MDT discussion, and timely intervention is required for lesions suspicious of malignancy. There are no uniform recommendations for the management of mixed tumors of the lung, and an individualized treatment plan needs to be developed based on the patient’s actual condition.