Introduction: Ameloblastic fibro-odontoma (AFO) is a relatively uncommon mixed odontogenic tumor usually detected in the early years of life. Common signs and symptoms include asymptomatic swelling, delayed tooth eruption, and a radiographically distinct lesion that appears radiolucent-opaque with well-defined borders. The standard treatment for AFO involves enucleation of the tumor, accompanied by long-term follow-up to monitor for potential recurrence or complications.

Patient case summary: A 10-year-old girl was referred to the Oral Medicine and Radiology department due to a small swelling in the lower right back teeth area, present for 5 to 6 months. A CBCT scan revealed a large, well-defined, cauliflower-like radiopaque lesion in the right ramus region. The lesion was enucleated under general anesthesia, and histopathological analysis showed focal islands of odontogenic epithelium with low cuboidal to columnar cells, along with ameloblastomatous proliferation, calcifications, and dentinoid tissue.

Discussion: Mixed odontogenic tumors are a group of rare lesions that can lead clinicians to consider a variety of differential diagnoses; therefore, it is essential to conduct thorough clinical and radiological investigations, perform proper surgical excision, achieve an accurate histopathological diagnosis, and ensure long-term follow-up in order to develop and implement an appropriate treatment plan for the patient.