A 43-year-old woman with a history of Sjögren's syndrome was admitted to our hospital because of dyspnea due to anemia. Her chest X ray film on admission revealed an anterior mediastinal tumor. We diagnosed this tumor as a thymoma based on a chest CT scan and magnetic resonance imaging. Because the latter indicated that the thymoma may have involved the mediastinal great vessels, preoperative chemotherapy with cisplatin, doxorubicin hydrochloride, vincristine sulfate and cyclophosphamide. Thereafter the tumor was successfully resected along with part of the right pleura. Thymoma as a complication of Sjögren's syndrome is rare. Here we also discuss the treatment of thymoma and the combination of these two diseases.