When dealing with tumors of the thymus and mediastinum, both the surgeon and the pathologist are confronted with a vast variety of different pathohistologic entities, among which epithelial tumors, namely thymomas and thymic carcinomas, are the most frequent. Intimate knowledge of the different entities and their anticipated clinical outcome is required to meet diagnostic and therapeutic challenges. We herein briefly update the reader with the most important new changes in the 2004 WHO classification of tumors of the thymus and to important new trends in the treatment of some of these tumors.