**Abstract**

Ameloblastoma is a benign odontogenic tumor of epithelial origin with locally aggressive behavior. It affects a broad age range of patients and it is most commonly found in the mandible, especially posterior area. The majority of ameloblastomas are conventional (multicystic), which are more difficult to eradicate than the unicystic or peripheral types. Although most of ameloblastoma cases can be treated predictably with radical surgical treatment, the management of recurrent and metastasizing ameloblastomas remains a major challenge. Surgical treatment is standard, but the extent of resection is controversial. Radical resection with segmental and marginal mandibulectomy or curettage and enucleation with better quality of life, but with higher recurrence rate. Besides the conventional surgical treatment, novel therapy options like neoadjuvant molecular targeted therapy and decompression in young patients could make a significant improvement in the management of the disease. The aim of this chapter was to determine the present and future concepts of treatment and discuss significant factors responsible for recurrence.

**Keywords:** ameloblastoma, odontogenic tumors, surgical procedures, molecular targeted therapy, recurrence
