Carcinoma ex pleomorphic adenoma is transformed at the incidence of 1-20% in pleomorphic adenoma and frequently recurred. It accounts for 10% of all malignant salivary tumors and its average age of occurrence is 60s. It will present in a large, long-standing pleomorphic adenoma or in one that was previously treated but has recurred. According to cell composition in malignant cell carcinoma, and clear cell adenocarcinoma. Most (75%) occur in parotid gland, while about 20% occur in the minor gland of the oral mucosa. The metastasis rate to regional lymph node is about 25%, and to distant organs about 33% and the 5-year survival rates are 40%. Though the treatment of the carcinoma ex pleomorphic adenoma is not established, it is treated ideally with and extensive resection, neck dissection, postoperative radiotherapy, and chemotherapy. When occurred in parotid gland, facial paralysis is reported. With a review of literatures, we report a case of carcinoma ex pleomorphic adenoma which operated with total parotidectomy and supraomohyoid neck dissection.