A 66-year-old male presented with dyspnea and cough for 1 month. Fiberoptic bronchoscopy disclosed a huge tumor over the posterior wall of the trachea 1 cm below the vocal cord and biopsy pathology was diffuse, small cleaved lymphoma. Primary malignant lymphoma of the trachea is extremely rare. Its clinical course varies widely, and has no accepted standard of management. We describe herein the whole clinical course of this patient, which is not similar to previous reports. The relation of primary tracheal lymphoma to mucosa-associated lymphoid tissue (MALT) is also discussed.