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Primordial odontogenic tumor (POT) has been included as a new entity in the latest WHO classification (2017). Only 17 cases have been documented worldwide, and here, we report the eighteenth case – the second in Europe and first in Poland. The study describes a case of a 16-year old boy, diagnosed with primordial odontogenic tumor. The patient was treated with enucleation and there was no recurrence in the follow-up period. Due to the small number of cases, further reports are crucial to obtain pathomorphological and clinical characteristics of primordial odontogenic tumor