The calcifying epithelial odontogenic tumor known as Pindborg's tumor, is a rare odontogenic neoplasm of the jaws. One of their characteristics is the cortical expansion and the relationship with a non erupted tooth. Since the original description in 1955, only 200 cases approximately have been described in the world literature. This article reviews the literature and describes a case of patient who presented calcifying epithelial odontogenic tumor in the jaw undergoing surgical excision treatment with an evolution without complications.