Ameloblastic Fibro-Odontoma of the Maxilla in a 4-Year-Old Girl:A Rare Pediatric Case

Ameloblastic fibro-odontoma (AFO) is a rare mixed odontogenic tumor of childhood whose biological nature remains debated. Recent World Health Organization (WHO) classifications consider most lesions previously diagnosed as AFO to fall within the spectrum of developing odontomas rather than representing distinct odontogenic neoplasms.We report the case of a 4-year-old girl who presented with progressive left cheek swelling. Imaging revealed a well-circumscribed mixed radiolucent–radiopaque lesion of the anterior maxilla, associated with displacement of developing tooth germs. Complete surgical enucleation was performed. Histopathological examination demonstrated a biphasic odontogenic tumor composed of cords and nests of ameloblastoma-like epithelium embedded in an immature ectomesenchymal stroma resembling dental papilla, with associated deposition of dentinoid and enamel matrix, consistent with ameloblastic fibro-odontoma. No recurrence was observed at 2-year follow-up.This case underscores the central role of careful histopathological evaluation in the diagnosis of mixed odontogenic tumors and supports the concept that a subset of these lesions may represent true odontogenic neoplasms.