Purpose of the Review <p>Assess new options and best sequence or combination strategies for the treatment of metastatic thymic epithelial tumors.</p> Recent Findings <p>Besides historical cytotoxic chemotherapy regimens, which remain standard-of-care for many patients with thymoma, new options include antiangiogenic agents and immune checkpoint inhibitors (ICIs) in the first-line setting combined with carboplatin and paclitaxel for thymic carcinoma. Antiangiogenic agents are also used in the second-line setting, possibly sequenced or combined with ICIs. With the latter, comprehensive assessment for autoimmune disorders is advised, with subsequent close clinical and biological monitoring. Precision medicine strategies may be implemented with comprehensive genomic profiling and use of targeted agents.</p> Summary <p>Multidisciplinary tumor board is key to optimize the treatment pathway for patients with metastatic thymic epithelial tumors, with a need for prospective studies assessing the best combination strategies.</p>