Thymic epithelial tumors, a malignancy originating in the thymus, are the commonest primary neoplasm of the anterior mediastinum; however, among thoracic tumors, they have a relatively low incidence rare. Thymic epithelial tumors can be broadly classified into thymic carcinoma and thymoma. As the cornerstone of thymic tumor treatment, surgery is the preferred treatment for early-stage patients, whereas, for advanced unresectable thymic tumors, the treatment is chemoradiotherapy. Targeted therapy is less effective for thymic tumors. Moreover, the use of immune checkpoint inhibitors as another effective treatment option for advanced unresectable thymic tumors, particularly thymomas, is limited owing to immune-related adverse effects. Here, we have summarized all pertinent information regarding chemotherapy, especially preoperative neoadjuvant chemotherapy, and chemotherapy in combination with other treatments, and reviewed the effectiveness of these procedures and recent advances in targeted therapy. In addition, we analyzed the efficacy and safety of immune checkpoint inhibitors in thymic epithelial tumors, to provide a holistic treatment view.Copyright © 2024. Published by Elsevier B.V.