The primordial odontogenic tumor (POT) is a rare mixed odontogenic tumor composed of mesenchymal cells, columnar odontogenic epithelium, and fibromyxoid stroma affecting the posterior mandible of children and adolescents. Herein, we report 3 patients with POT and the clinicopathological features of POT previously reported in the literature. A 12-year-old, 19-year-old, and 4-year-old patient presented an asymptomatic swelling in the posterior maxilla and posterior mandible. Imaging exams revealed radiolucent lesions associated with unerupted teeth. The lesions were surgically removed, and the histopathological examination revealed spindle-to-ovoid mesenchymal cells in a fibromyxoid stroma surfaced by columnar odontogenic epithelial cells with reverse nuclear polarization. Deposition of mineralized tissue was observed. The final diagnosis was POT, and patients did not exhibit signs of recurrence. POT should be included in the differential diagnoses of intraosseous lesions in the posterior mandible in pediatric patients.
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