A 49-year-old female presented with cough and intermittent breathlessness for one week. Chest CT and fiberoptic bronchoscopy revealed multiple tumors in the lower trachea. The patient had been diagnosed with unclassified autoimmune disease in 1993. She also had a past history of gastric mucosa-associated lymphoid tissue lymphoma (MAL Toma) with recurrence, and had received two courses of chemotherapy with durable remission. We treated her tracheal tumors based on the previous lymphoma history, without tissue proof, and she reached complete remission. In this report, we review the characteristics of tracheal or bronchus-associated lymphoid tissue lymphomas (BAL Toma), as evidence for accepting the possibility of tracheal lymphoma in our case.