pISSN 1738-1843 eISSN 2092-8920 © 2013 The Korean Society of Pathologists/The Korean Society for Cytopathology This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/ by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. Thymolipoma is a rare anterior mediastinal tumor composed of mature adipose tissue and benign thymic tissue, and is a benign neoplasm for which complete surgical excision is curative. Literature has reported that the thymic parenchyma of thymolipoma exceptionally contain myoid cells that are immunoreactive for desmin, muscle-specific actin and myoglobin. Herein, we describe a very rare case of thymolipoma with myoid cells. To the best of our knowledge, this is the sixth case of thymolipoma containing striated myoid cells in the English literature since 1972.