Myoepithelial carcinoma or malignant myoepithelioma are considered extremely rare malignant salivary gland neoplasms. Their clinical behaviour by literature seems variable: most studies reported good prognosis for low-grade malignancy and vice versa. Nevertheless long term survival has been described in patients with high-grade tumours, while patients with low-grade have been reported to develop metastases. We report a case of myoepithelial carcinoma of the parotid gland arising in pleomorphic adenoma in a 52-year-old woman with favourable course in long term follow-up.