Thymic epithelial tumor（TET）represents a rare malignant mediastinal neoplasm with limited systemic treatment options for advanced - stage patients. In recent years，immunotherapy has been widely applied in solid tumors such as lung cancer and melanoma. Clinical studies of programmed death - 1/programmed death - ligand 1（PD - 1/PD - L1）inhibitors in TET have also demonstrated preliminary efficacy. However，immunotherapy in TET is associated with specific and highly prevalent immune -related adverse events（irAE），severely limiting its clinical application. With advances in understanding the pathophysiology of irAE and the adoption of multidisciplinary management approaches，early recognition，tiered interventions，and personalized treatment strategies have progressively matured. The implementation of these systematic management protocols provides critical safeguards for the safe advancement of immunotherapy in TET and holds promise for further improving patient survival outcomes. This article provides a systematic review of research progress in immunotherapy for TET，the potential mechanisms underlying irAE，and comprehensive management strategies.