Background. Carcinosarcoma or true malignant mixed tumor of the parotid gland is extremely rare, accounting for <1% of all salivary gland malignancies.

Methods. A 63-year-old woman presented with a 5-cm right parotid mass which was resected with a radical parotidectomy and infratemporal fossa dissection.

Results. The tumor contained two distinctive histologic patterns, that of a poorly differentiated ductal carcinoma and a pleomorphic rhabdomyosarcoma. Additionally, a residual focus of pleomorphic adenoma was present peripherally, Immunohistochemical and electron microscopic studies confirmed the skeletal muscle differentiation.

Conclusions. The pattern of combined rhabdomyosarcoma and ductal carcinoma has not been previously reported and adds further evidence to the myoepithelial derivation of these tumors. © 1994 John Wiley & Sons, Inc.