A 36-year-old woman was examined and treated for a rare odontogenic tumor in the mandible, the calcifying epithelial odontogenic tumor (CEOT), also known as the Pindborg tumor. The tumor extended from the left mandibular molar region to the right premolar area and had almost completely destroyed the bone in the anterior mandibular region. An orthopantomogram showed a multiloculated radiolucent lesion, with an unerupted tooth in the center surrounded by a radiopaque area. In addition, finely granular calcification was visible here and there in the tumor area. In the histologic examination the tumor tissue could be identified as calcifying epithelial odontogenic tumor. Under electron microscopy the fibrillar structure at the basal cell level suggested the presence of amyloid. The tumor was removed by enucleation with a collar of surrounding tissue. No signs of recurrence have been noted at follow-up examinations.