The purpose of this article is to review the pertinent literature and discuss the optimal treatment and outcomes for patients with ameloblastoma. Ameloblastoma is an uncommon benign, locally aggressive odontogenic neoplasm that usually occurs in the vicinity of the mandibular molars or ramus. Uncontrolled, ameloblastoma may cause significant morbidity and occasionally death. The median age is approximately 35 years and males and females are equally affected. The majority of ameloblastomas are multicystic, which are more difficult to eradicate than the unicystic and peripheral varieties. Although surgery is the mainstay of treatment, the extent of resection is controversial. Radical resections, including marginal and segmental mandibulectomy, result in local control rates exceeding 90%. In contrast, conservative procedures such as enucleation and/or curettage result in local control rates of approximately 80% and 50% for unicystic and multicystic ameloblastomas, respectively. Limited experience with radiotherapy indicates that it may reduce the risk of progression and result in long-term local control in the occasional patient with incompletely resectable disease. The optimal treatment for ameloblastoma is wide en bloc resection. Radiotherapy may improve the likelihood of local control in the occasional patient with incompletely resectable tumor.