Primary lymphomas of the larynx are extremely rare. Even more unusual is their association with rheumatologic diseases. Some cases of lymphoma have developed after chemotherapy for rheumatologic diseases, suggesting that the treatment may contribute to the development of lymphoma. The converse can also occur-the development of rheumatologic disease after a primary laryngeal lymphoma-but that is even rarer. We report a case of primary extranodal marginal zone B-cell MALT (mucosa-associated lymphoid tissue)lymphoma of the larynx in a 42-year-old woman treated with involved-field radiotherapy who developed systemic lupus erythematosus (SLE) 1 year after the lymphoma diagnosis. Currently, the patient remains in remission 8 years after her diagnosis. The SLE, for which she is receiving maintenance therapy, is stable.