Metaplastic thymoma is a rare primary thymic epithelial tumor, and its biologic behavior is uncertain. We report 7 cases of metaplastic thymoma. The spindle components of the tumor seemed to be undergoing epithelial-mesenchymal transition, which is characterized by the loss of E-cadherin expression. Epstein-Barr virus seemed not to have an etiologic role in metaplastic thymoma. No gross genetic alterations were found by using comparative genomic hybridization. The patients were alive and well in follow-up ranging from 11 to 172 months (mean, 81.7 months). The findings suggest that metaplastic thymoma has a benign clinical course. Unless the histomorphologic findings show malignant features, surgical excision alone is recommended.