Carcinoma in pleomorphic salivary adenoma is very difficult to diagnose preoperatively. Fine needle aspiration cytology had a disappointingly low sensitivity for this tumor, potentially misdirecting surgical management. While good locoregional disease control could be achieved with surgery and radiotherapy, carcinoma in pleomorphic salivary adenoma was shown to be aggressive with a high disease-specific rate of mortality. Given that incomplete tumor resection was the most important prognostic factor, a high index of clinical suspicion, radical ablative surgery, and immediate soft-tissue and nerve reconstruction for proven cases are advocated.