Introduction: Clear cell neoplasms of the jaw are very infrequent and a review of the literature reports only isolated cases of metastatic renal clear cell carcinoma of the jaw.
Case presentation: A 68-year-old man presented with an osteolytic lesion of the left hemimandible. The first diagnostic hypothesis was a third molar follicular cyst. Surgical treatment consisted of enucleating the lesion preserving the alveolar nerve and extracting of the retained tooth. Unexpectedly, the lesion presented as a solid.
Conclusion: The authors report a case of a clear cell neoplasm involving the jaw in which histopathological exam presented an indeterminate histology. The histological characteristics of this tumor make it unique in the international literature.