Abstract Ameloblastoma is a rare odontogenic neoplasm involving the mandible and maxilla, with multiple histologic variations , and high chances of recurrence rates if treated improperly. The current standard of treatment is wide local excision with appropriate margins and immediate reconstruction. Here we have reviewed the ameloblastoma literature, using the available evidences to highlight the changes in management over the past several years. Additionaly, we explore the recent molecular characterization of these tumors which may direct towards new potential avenues of personalized treatment.