The present paper discusses the case of a patient who was surgically treated for a rare calcifying epithelial odontogenic tumour (Pindborg tumour) at the Department of Maxillofacial Surgery, F. Chopin Clinical Voivodeship Hospital in Rzeszow. Calcifying Epithelial Odontogenic Tumour (CEOT) is a benign odontogenic tumour arising from the remnants of the dental lamina epithelium. The first three cases of this tumour were recognized by the Danish pathologist J.J. Pindborg in 1955. Since then Calcifying Epithelial Odontogenic Tumour has been commonly referred to as the Pindborg tumour. This type of neoplasm is relatively rare, since it occurs in approximately 0.4% of all odontogenic tumour cases. Due to a fairly common tendency of recurrence, estimated to be approximately 14% of all cases, the preferred o choice of treatment is radical surgical procedure and postoperative follow-up. Appropriate clinical and histopathological diagnosis is very important before applying the most suitable surgical treatment. Based on the case reviewed and the available literature, we can confirm the suitability of the therapeutic procedure course aligned with contemporary views, guidelines and established standards.