Desmoplastic ameloblastoma is a rare variant of ameloblastoma and only 54 cases have been previously reported in the English literature. We report ten cases of desmoplastic ameloblastoma of the jaw observed between 1972 and 1998 and discuss the pathogenesis of the tumor. Eight of the ten tumors were located in the anterior region of the jaw. Radiographically, six cases showed mixed radiolucent/radiopaque appearance with ill-defined margins and the other four cases lacked typical findings of ameloblastomas. The tumors were located mainly in the alveolar region and occupied the tooth-bearing area, without tooth resorption or impacted teeth. Histologically, the desmoplastic ameloblastomas were characterized by extensive stromal desmoplasia, and small tumor nests of odontogenic epithelium were scattered in the stroma. Oxytalan fibers stained by potassium monopersulfate-aldehyde fuchsin were demonstrated in the stroma of all desmoplastic ameloblastomas. From these findings, we presumed that the desmoplastic ameloblastomas might have developed in the periodontal membrane.