Primary intraosseous carcinoma (PIOC) is an extremely rare lesion, almost always occurring in cranial bones. The origin of this tumor, specific to the maxillae, is associated with the cells of the epithelial rests of Malassez. Among the histotypes which can be included in these neoplasms, verrucous carcinoma is of particular interest due to its rarity: only a single case has been reported to date. After a short survey of the literature, the authors describe a directly observed case of verrucous carcinoma arising from a maxillary odontogenic cyst.