Dentinogenic ghost cell tumor (DGCT) is a locally invasive neoplasm that forms ameloblastomalike epithelial islands associated with dyskeratosis of ghost cells and formation of atypical dentin. It is reported that DGCT has a high recurrence rate. We report a case of DGCT arising in the maxilla. A 42-year-old man was referred to our department with a chief complaint of a palatal swelling in the maxillary anterior tooth region. On radiographic examination a well-circumscribed radiolucent lesion with nonhomogeneous calcifications was seen around the root of the right maxillary lateral incisor. The tooth showed vital pulp. The preoperative clinical diagnosis was a maxillary tumor, and the tumor was enucleated under local anesthesia. The histopathological diagnosis was DGCT. Postoperative healing was uneventful, and there was no sign of recurrence during 18 months of follow-up.
