Primordial odontogenic tumor (POT) is a rare, benign, mixed epithelial–mesenchymal neoplasm, histologically characterized by spindle- to stellate-shaped cells in a fibromyxoid stroma resembling dental papilla, bordered by columnar epithelium. It occurs mainly in children and adolescents, often in the posterior mandible, and radiographically presents as a well-defined radiolucency associated with unerupted teeth. An 18-year-old male presented with asymptomatic swelling of the right mandible. Imaging revealed a well-demarcated unilocular radiolucency extending to the ascending ramus with cortical thinning and perforation. Under a provisional diagnosis of ameloblastoma, right mandibulectomy with plate reconstruction was performed. Histology showed spindle and stellate cells in a delicate collagenous stroma lined by columnar epithelium with reverse nuclear polarity and areas of solid and cystic epithelial proliferation, confirming POT. We report the first case of POT in Saudi Arabia, managed surgically with no recurrence at seven-year follow-up. This case highlights the diagnostic challenges of POT and the importance of continued case documentation to guide recognition and management.
Almazyad et al. (2026) studied this question.