Tehran University of Medical Sciences Journal of Craniomaxillofacial Research 2345-5489 4 2 2017 07 22 372 376 EN Abbas Karimi Craniomaxillofacial research center, Tehran University of medical sciences department of oral and maxillofacial surgery, Tehran University of medical sciences. Mahboube Hasheminasab Department of Oral & Maxillofacial Surgery, Faculty of Dentistry, Tehran University of Medical Sciences, Iran. Samira Derakhshan Department of Oral and Maxillofacial Pathology of dentistry school, Tehran University of Medical Science, Tehran, Iran. 2017 07 22 2017 07 22 Carcinosarcoma of salivary gland is a rare and aggressive tumor composed of both carcinomatous and sarcomatous components which metastasize together. Most tumors arise in parotid gland from a preexisting pleomorphic adenoma. Methods and Results: We report a case of carcinosarcoma of minor salivary gland of lower lip with subsequent infiltration to mandible and pelvic metastasis after recurrence. The patient was a 38 year old man who underwent surgical resection and adjuvant chemo radiotherapy and demonstrates recurrence after 9 months with metastasis to left iliac fossa. Conclusion: Carcinosarcoma of minor salivary gland is an extremely rare malignancy with poor prognosis and is best managed by combination of surgical resection and postoperative radiation. Key words: Carcinosarcoma, Salivary gland, Metastasis, Recurrence. https://jcr.tums.ac.ir/index.php/jcr/article/view/158 https://jcr.tums.ac.ir/index.php/jcr/article/download/158/185