Objective: To explore the clinicopathological features, diagnosis and differential diagnosis of tracheal pleomorphic adenoma. Methods: We observed the clinical data, histopathology, immunohistochemistry of a case of tracheal pleomorphic adenoma, and reviewed some relevant literatures. Results: The tumor was smoothing appeared as the surface, irregular nodular, measuring 2.5 cm × 1.5 cm × 1.0 cm in size. The tumor was gray-white on cut surface, soft to rubbery translucent, and couldn’t be observed obviously bleeding and necrosis. Under light microscope, the mass was composed of epithelial and myoepithelial cells. The epithelial cells were nest-shaped, cord-like and beam in the chondromyxoid stroma. The epithelial cells had normochromic nucleus with eosinophilic cytoplasms and had glandular distribution in some parts. There was no definitive cellular pleomorphism and mitotic figures. Immunohistochemically, cytokeratin, S-100 protein, and p63 of myoepithelial cells expressed positively. Cytokeratin, cytokeratin 19 and cytokeratin 7 of epithelial cells were positive. Conclusion: Pleomorphic adenoma of the trachea should be included in the differential diagnosis of tracheal tumors, so as not to misdiagnosis, although the occurrence is very rare. Future studies with additional cases are needed in order to define the clinical course, biologic behavior and prognosis of pleomorphic adenoma of the trachea.