Thymomas composed predominantly of epithelioid tumor cells with scattered lymphocytes have been well recognized in the literature. This subtype of thymoma has been variously termed epithelial-rich thymoma, well-differentiated thymic carcinoma, atypical thymoma, or World Health Organization (WHO) type B3 thymoma. Regardless of the designation however, these tumors are known to show a spectrum of histopathological growth patterns that may pose challenges in interpretation and diagnosis, particularly when dealing with small mediastinoscopic biopsies. Just like any other type of thymoma, those composed predominantly of epithelioid cells may present as encapsulated or invasive tumors. Nevertheless, compared to other subtypes of thymoma, they are uncommon neoplasms. Therefore, it becomes very important to sufficiently sample thymomas before making a diagnosis of a particular subtype, especially when the tumor is rich in epithelioid cells and only has a scant lymphocytic component. Because of the unusual occurrence of these tumors, there are only few large series of cases that attempt to highlight not only the more salient histopathological features but also the most important immunohistochemical and molecular characteristics.