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Introduction: ameloblastomas are benign locally invasive epithelial odontogenic tumors arising from remnants of the dental lamina and odontogenic epithelium. Case presentation: a 39-year-old female patient presenting with progressive and painless swelling of the right side of the face and neck. A well-defined non-tender, firm, non-fluctuating tumor measuring 5 cm in diameter was identified upon palpation. A panoramic radiography showed an unilocular radiolucent lesion near the first right mandibular molar tooth. These findings were confirmed by computed tomography. The biopsy revealed a cyst cavity lined by fine odontogenic epithelium. Patient underwent a total segmental mandibulectomy.Discussion: ameloblastoma is a rare lesion with features of maxillary cysts. Histologic and immunohistochemical findings allow distinguishing ameloblastomas from other benign and malignant lesions. Conclusion: it is a benign slow-growing tumor with a high capacity for recurrence. The recommended therapeutic approach is complete surgical resection with a wide margin of normal tissue. Extended follow-up is advisable due to its high recurrence and malignant transformation potential.
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