Abstract
Ameloblastic carcinoma is a rare malignant epithelial tumor, mostly affecting the mandible. Maxillary ameloblastic carcinoma is even more uncommon and its clear cell variant has been exceedingly rarely described. Because clear cell lesions of jaw bones are both rare and diagnostically challenging, the incidence of the tumor, as well as the criteria for classification are not precisely defined. Differential diagnosis includes a variety of benign or malignant tumors of odontogenic and salivary gland origin, as well as metastatic tumors. Diagnostic dilemma extends to therapeutic approach. Treatment modalities are still debated. The present case report discusses the clinical and histologic differential diagnosis of clear cell variant of an aggressive maxillary ameloblastic carcinoma which was successfully treated with preoperative radiation therapy, followed by surgical excision of the residual lesion and reconstruction of the maxilla.