Objectives: The ameloblastic fibro-odontoma (AFO) is a rare, slow-growing, odontogenic tumour. This benign neoplasm has been defined by the World Health Organization as “a neoplasm composed of proliferating odontogenic epithelium in a cellular ectomesenchymal tissue with varying degrees of inductive changes and dental hard tissue formation”.

Case: An eight-year-old male patient presented to a private dental clinic with the complaint of tooth deficiency in the posterior of the right mandible and he was directed to our faculty due to the lesion in the orthopantomography (OPG). The patient did not have any complaints such as pain or swelling. An extraoral asymmetry was not observed in the patient whose gingival hyperplasia was detected in the relevant region during intraoral examination. In the cone-beam computed tomography (CBCT) scan, which was taken for detailed evaluation, expansive lesion that did not cause perforation in the buccal and lingual bone cortex was observed. A well-defined sclerotic border and irregular calcified tissue mass and impacted tooth-containing radiolucent lesion were detected in the right mandibular premolar region. As a result of histopathological examination of the operated lesion, the diagnosis of 'ameloblastic fibro-odontoma' was made. The patient was recommended to come for 6-month controls.

Conclusion: The differential diagnosis of AFO should also include lesions with mixed radiographic patterns, such as calcifying epithelial odontogenic tumor, calcifying odontogenic cyst and possibly adenomatoid odontogenic tumor. Treatment of AFO is generally enucleation with concurrent removal of associated unerupted tooth. As the tumor is well encapsulated, there is little tendency to local invasion.