Ameloblastic carcinoma is a rare type of ameloblastoma that has received little mention in the literature. While a number of cases have been published over many years, no institution has been able to produce a substantial case series. Ameloblastic carcinoma originates in the embryonic tooth components. It is believed to be an aggressive tumor that can metastasize; once metastasis occurs, the prognosis tends to be poor. Ameloblastic carcinoma is primarily a surgical condition that is best treated with resection; there has been little indication that other modalities are helpful. We present the case of a 40-year-old woman who was found to have a mandibular lesion by a dentist. After surgical resection, the tumor was found to be an ameloblastic carcinoma. The patient recovered without complication, and she was recurrence-free 18 months postoperatively. We also briefly review the available literature on the natural history of and management options for this rare tumor.
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