Malignant pleomorphic adenoma arising in the trachea has not been reported in the literature. We report here a case of malignant pleomorphic adenoma (malignant mixed tumor) occurring in the trachea of a 65-year-old woman. The tumor metastasized to the lung and the chest wall 11 years after complete resection of the primary tumor, which was a polypoid submucosal tumor, 1.3 cm in diameter. Light microscopic examination of the primary and metastatic tumors showed the presence of epithelial and stromal elements, consisting of grandular structures, foci of squamous metaplasia and a myxochondroid stroma. Many tumor cells showed myoepithelial cell features by electron microscopy, and immunoreactivity for S-100 protein and GFAP was also seen in many of them. These findings were consistent with those of pleomorphic adenoma. However, the epithelial elements were cytologically atypical with prominent mitotic figures. Infiltration of the tumor cells into the surrounding soft tissue was also seen. No foci of benign pleomorphic adenoma were found in the primary tumor. These findings indicate that this tumor was not a carcinoma ex pleomorphic adenoma, but a true malignant pleomorphic adenoma (true malignant mixed tumor) of the trachea.