Abstract

Ameloblastic fibro-odontoma is a rare mixed odontogenic tumor, contains variable amounts of dental hard tissues. It is a controversial lesion. 
Previously, it was considered as an individual tumor, but recently, the World Health 
Organization (WHO) has defined it as a developing odontoma. It has histologic features of both ameloblastic fibroma and complex odontoma.
Ameloblastic fibro-odontoma occurs more frequently in the posterior 
mandible of children and young adults.
This case report presents an extensive developing odontoma or Ameloblastic fibro-odontoma in a 4-year-old boy, positioned in the posterior maxilla; that is a rare region for its occurrence. Facial asymmetry and intraoral swelling were apparent. There was also excessive displacement of permanent first and second molar teeth of the upper jaw to the floor of the orbit. Surgical removal of the lesion and the associated teeth was indicated for treatment. No recurrence was observed after 2 years of follow-up.
Management of an expansile ameloblastic fibro-odontoma with maxillary 
sinus involvement is challenging. Cone beam computed tomograghy (CBCT) is helpful for more detailed investigation.

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