A case of malignant mixed tumor of the trachea in a 56-year-old man is described. His tumor was removed by segmental tracheal resection, and end-to-end anastomosis was performed. Histologically, the tumor was characterized by a biphasic composition showing admixtures of epithelial elements in varying proportions; these were cytologically atypical with prominent mitotic figures. However, stromal elements were osteoid and mixoid with a benign appearance. The patient had no evidence of disease in the head and neck region and had no history of previous surgery for a salivary gland tumor. These findings were interpreted as indicative of malignant pleomorphic adenoma of the trachea.