The ameloblastoma is a benign neoplasm of epithelial odontogenic origin, slowly growing, locally invasive and it is the most common odontogenic tumor in the jaws. Histologically, various types have been described in literature. The desmoplastic variant is rare and characterized by difference in the typical findings of ameloblastoma, including localization, radiographic and histological features. The purpose of this article is to report a case of desmoplastic ameloblastoma in the left maxilla, and review of relevant literature, emphasizing peculiar aspects of this unusual lesion.