Here we describe a case of API2-MALT1 fusion-positive MALT lymphoma of the bilateral submandibular glands with no evidence of autoimmune syndromes….He was treated with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone. After 6 years, the patient is alive and disease free. In the present case, the patient with API2-MALT1 fusion-positive MALT lymphoma had a good outcome despite the advanced clinical stage.