Ameloblastoma, a rare odontogenic tumor that has fascinated clinicians and researchers for decades. This review delves into the tumor's origin, deeply investigating the etiopathogenesis rooted in the odontogenic epithelium associated with enamel formation. It elucidates key cellular and molecular mechanisms driving its development, shedding light on the intricate factors governing initiation and progression. Furthermore, the review focuses on the management of ameloblastoma, with an emphasis on surgical interventions. This review also addresses the vexing issue of tumor recurrence and the potential complications that can arise during and after treatment. By examining these issues in detail, it offers essential insights into effective strategies for managing these aspects of ameloblastoma, ensuring that clinicians and researchers are well-equipped to navigate the complexities of this unique odontogenic tumor. In summary, this review offers a holistic and in-depth exploration of ameloblastoma, from its origins to its management and the challenges it presents to the medical community.