Abstract
Ameloblastoma is rare and the most aggressive and locally invasive odontogenic tumor derived from odontogenic epithelium with a tendency for recurrence and bone destruction. We report a case in which the initial symptom of nevoid basal cell carcinoma syndrome （NBCSS） was thought to be due to ameloblastoma. The patient was a 15-year-old male with no medical history or family history. The ameloblastoma of the mandible was removed; at 6 years after the operation, a dentigerous cyst developed in the left maxilla; at 9 years, an odontogenic keratocyst developed in the right maxilla; at 12 years, no recurrence was observed and the patient is being followed up. In NBCCS, which presents with various symptoms, the pathophysiology is not limited to major symptoms such as odontogenic keratocysts even in the maxillofacial region. It is important to deepen understanding of NBCCS diseases and make efforts for early diagnosis.
