Dentinogenic ghost cell tumor (DGCT) is considered as a neoplastic counterpart of the calcifying odontogenic cyst (COC). beta-catenin mutations have been described in COC suggesting a critical role in its histogenesis. In this study, we report a patient with DGCT contains a missense mutation on codon 3 (ACT --> TCT) of beta-catenin gene. Immunohistochemistry showed nuclear, cytoplasmic, and membranous accumulation of beta-catenin in the tumor cells. TUNEL assay showed positive signals in nucleated cells adjacent to the ghost cells. Our data suggest that beta-catenin plays an important role in the tumorigenesis of DGCT. DGCT may develop by an improper differentiation process coordinated by Wnt signaling pathway. Further studies are needed to determine the genotypic/phenotypic characteristics of ghost cell-containing odontogenic lesions.