In 1983, Damm and associates [1] described an epithelial odontogenic tumor that contained areas that were diagnostic for adenomatoid odontogenic tumor (AOT) and calcifying epithelial odontogenic tumor. The tumor was termed ‘‘combined epithelial odontogenic tumor’’; since the initial description, a small number of case reports have appeared in the literature [2–8]. In 1993, Ledesma and associates [9] published a report of 12 cases of AOT with calcifying epithelial odontogenic tumor (CEOT)-like areas. They concluded that these additional features represent a frequent histomorphologic pattern of AOT and that the combination should not be considered to be a separate entity. In 1996, Miyake and associates [8] studied the ultrastructure of an example and concluded that electron microscopy changes were typical of AOT and CEOT. The epidemiology of combined epithelial odontogenic tumor closely approximates that of adenomatoid odontogenic tumor. It is most commonly seen in the second and third decades of life and there is a female predilection. The anterior regions of the jaws—more often the maxilla—is the most common location and usually it is associated with the crown of an unerupted or impacted anterior tooth. Radiographically, it typically is seen as a well-defined unilocular radiolucency that may contain radiopacities (Fig. 1). The microscopic features consist of an encapsulated epithelial proliferation that is composed of