Myoepithelial carcinoma, arising in a recurrent or in a pre-existing pleomorphic adenoma of the parotid gland is an extremely rare cancer. We herein report the case of myoepithelial carcinoma occurring in a recurrent pleomorphic adenoma, which showed a high metastatic potential. A 53-year-old male, who had undergone a superficial parotidectomy of the pleomorphic adenoma 2 years previously, presented with recurrent parotid swelling and with multiple coin lesions in the lung. A total parotidectomy and a thoracoscopic biopsy of the lung lesion revealed both lesions to be myoepithelial carcinoma. The patient died about 12 months later despite undergoing intensive chemotherapy.