Abstract. The keratoameloblastoma is a benign lesion of the jaws, which is a rare histologic variant of the ameloblastoma. There is a variation in the histopathologic appearance of reported cases under the appellation of keratoameloblastoma. The keratoameloblastma has usually keratin formation by the ameloblastomatous epithelium and varies in size. English literature reports only 14 cases of keratoameloblastma. We described an additional case of the tumor developed in the right mandible of a 26-year-old woman. It was presented as an expansile and radiolucent lesion. Histologically, solid tumor islands were seen with some microcystic space within a fibrous stroma resembling an ameloblastoma. In addidion, a hyperchromatic columnar basal cell layer and parakeratin within the microcyst simulating an odontogenic keratocyst