Calcifying cystic odontogenic tumor (CCOT) is an uncommon benign cystic neoplasm of the jaw that develops from the odontogenic epithelium. It is clinically characterized as a painless-slow-growing tumor that affects the maxilla as well as the mandible, and generally occurs in young adults in the third or fourth decade of life. Herein, we present the case of a 16-year-old Japanese boy who showed a CCOT in the maxillary sinus. Panoramic radiography showed a unilocular lesion in the left maxillary sinus. Computed tomography showed an approximately 5-cm well-defined unilocular expansile lesion with multiple radiopaque calcific specks, arising from the left maxillary alveolar ridge. The lesion was surgically removed, under general anesthesia, and the patient was followed up for 3 years after the surgery, and there have not been any signs of recurrence.