Clear cell odontogenic tumors are rare. Review of the literature showe
d 9 cases with a prominent clear cell component. These lesions have ex
hibited an aggressive behavior characterized by an infiltrative local
growth pattern, recurrence, or metastases. We report a case of an odon
togenic tumor that exhibited a biphasic pattern and was characterized
by lymph node involvement identical histologically to the primary tumo
r. We conclude that the presence of a clear cell component in an amelo
blastomatous tumor should be viewed as a sign of de-differentiation, a
nd that a malignancy with or without metastases should be considered a
nd ruled out in such cases.