**1. Introduction**

Odontogenic tumors are considered as relatively rare and destructive neoplasms of the jaw bones. They are derived from the remnants of odontogenic tissue and each odontogenic tumor represents the abnormality in odontogenesis [1].

Ameloblastomas belong to benign odontogenic tumors with locally aggressive behavior. Although the incidence of odontogenic tumors varies from 1% to 32% of all jawbone tumors, ameloblastoma, alongside odontoma, is the most common benign odontogenic tumor [2]. It is predominantly found in the mandible (up to 80%) and most patients diagnosed with ameloblastoma are aged between 30 and 60 years [3].

The current, 5th World Health Organization (WHO) classification from 2022 distinguishes five different types of benign ameloblastoma as described hereafter [4]. They most commonly manifest as slow-growing and asymptomatic swelling with the ability to expand and perforate cortical bone. Slow-growing character and lack

of symptoms are considered responsible for delayed diagnosis of the ameloblastoma which is an ongoing problem, especially in developing countries [3].

Throughout history, primary treatment was, and still is, surgical with controversial extent of resection [5]. Taking into consideration severe clinical implications with high recurrence rate it is of utmost importance to provide sufficient guidelines and standardize surgical approach. In addition, recent literature has provided us with breakthrough in the understanding of genetic mutations and signaling pathways crucial in ameloblastoma pathogenesis [6]. Thus, novel therapy options like neoadjuvant molecular targeted therapy could significantly contribute to the management of the disease.

This chapter will address evidence-based treatment options and contemporary concepts of managing ameloblastoma.
