Ameloblastoma of the jaw is an aggressive benign tumor of epithelial origin that has generally been treated surgically even for metastases. This article is a review of the literature and a report of a previously unpublished case. Despite earlier reports, it is suggested that the available data clearly indicate that it is a radiosensitive tumor. In this report, specific recommendations for radiation therapy are outlined, and guidelines for treatment planning, radiation dosage, and fractionation expected outcome and follow-up are given.