A male patient presented with an extraordinarily large calcifying epithelial odontogenic tumor (CEOT or Pindborg-tumor) that affected the maxilla. The disease became evident due to alterations in the facial aspect, in particular of the perioral region, caused by the expanding tumor. CEOT is characterised by the slowly growing mass of part of the jaws. Multilocular or extraosseous manifestations are extremely rare. Malignant transformation with metastases is rare. Radiography depicts characteristic, but not obligatory, areas of calcification inside the tumor. The surgical therapy for CEOT is complete local resection with safe margins. If tooth bearing parts of the jaws are affected, these teeth almost always have to be removed. The prognosis is excellent for overall survival. Local recurrences have rarely been reported but may be found even decades after primary treatment. Three years following surgical therapy there is no evidence of local recurrence. A long-term follow-up control is recommended.