Ameloblastoma is the most common aggressive benign odontogenic tumor of the jaws. The tumor is often asymptomatic, presenting as a slowly enlarging facial swelling. The physical presence of the tumor may cause symptoms such as pain, ulceration, loosening of teeth, or malocclusion. Ameloblastoma is a locally destructive tumor with a propensity for recurrence if not entirely excised. It is seen in all age groups, but the lesion is most commonly diagnosed in the third and fourth decades. The tumor is considered a rarity in the young age group. The treatment of ameloblastoma is still controversial and presents some special problems in children like the growth of the jaw, the different incidence, behavior and prognosis of the tumor in children, which make the surgical considerations different from adults. Some reports have encouraged conservative treatment for ameloblastoma in children.