An 11-year-old girl presented to our specialized department with her mother who reported pain and facial alteration in the last 2 months. Intraoral examination showed dental crowding between teeth #11 and #12 and discrete volume increase in this region. A panoramic radiograph revealed a radiolucent unilocular lesion, circumscribed containing foci of radiopaque material and promoting root divergence between teeth #12 and #11. Computed tomography showed vestibular and palatine cortices disruptions. The diagnostic hypothesis was calcifying epithelial odontogenic tumor. We performed an exploratory puncture biopsy (which was positive for citrine liquid), an enucleation of the lesion, and reconstruction with bone graft (Bio-Oss with Bio-Gide membrane). The histopathologic diagnosis was ameloblastic fibro-odontoma. The patient is under follow-up. This case underscores the importance of clinical, radiographic, and histopathologic features providing correct diagnosis and adequate treatment of the patients.
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