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Carcinoma ex pleomorphic adenoma of the submandibular gland with distant metastases. A case of metastatic carcinoma ex pleomorphic adenoma (PA) of the submandibular gland is reported. While reports of local recurrence of this cancer are numerous, few cases of distant metastases have been reported. We report a case of a 52-year-old male patient with a history of PA diagnosed ten years previous. The patient presented reporting that the mass had increased size and pain during the previous six months. Needle biopsy results of this mass were compatible with a poorly differentiated carcinoma of the salivary ducts. Radical surgery with wide right neck dissection was performed with curative intent. Three years post-surgery liver and bony metastases were detected. In conclusion, the potential for malignant transformation of PA demands close follow-up of younger patients.