Thymic epithelial tumors (TETs), including thymoma, thymic carcinoma and neuroendocrine tumors, are uncommon tumors that originate from the epithelial cells of the thymus. Nevertheless, despite their rarity, they represent the most common tumor type located in the anterior mediastinum. Therapeutic choices based on staging and histology may include surgery with or without neoadjuvant or adjuvant therapy represented by chemotherapy, radiotherapy or chemo-radiotherapy. For patients with advanced or metastatic TETs, platinum-based chemotherapy remains the standard first-line treatment; however, some new drugs and combinations are currently under evaluation. In any case, proper management of patients with TETs requires a multidisciplinary team approach to personalize care for each patient.