The dentigerous cyst is a common oral lesion arising as a developmental ano
maly during amelogenesis. In rare instances, the epithelial lining of these
cysts may give rise to squamous cell carcinoma (SCC). Fewer than 50 cases
of this rare entity have been reported in the world literature to date, We
present an additional case of SCC arising in a dentigerous cyst with a rati
onale for our treatment approach. In addition, we offer a review of the lit
erature and a review of the clinical, histologic, and radiographic findings
associated with this finding. We discuss epidemiologic data and treatment
options.