We report a clinical case of a 41-year-old female patient affected by a Pindborg tumor located in the left mandibular angle. The accidental detection of the lesion and its frequent relationship with an impacted tooth can difficult its differential diagnosis, mainly with an odontogenic cyst. In spite of the existence of a recurrence rate of 14% after conservative treatment, we think that a quality treatment would require the practice of a block excision including healthy bony margins.