Abstract:A 15-year-old male patient was referred for diagnosis and treatment of a radiographic lesion located in the anterior region of the maxilla. Clinically, the patient presented a prolonged retention of the element 63 and absence of the element 23. No swelling around the alveolar ridge was observed. The radiographic findings were characterized by few tooth-like radiopaque structures surrounded by a radiolucent halo around the impacted element 23. The clinical hypothesis was stablished as dentigerous cyst associated with compound odontoma. The excisional biopsy was performed keeping the impacted tooth in place, to which an orthodontic bracket was bond for posterior traction. The surgical piece was taken to histopathological examination and the final diagnosis was calcifying cystic odontogenic tumor (CCOT) associated with compound odontoma. The calcifying cystic odontogenic tumor (CCOT) is a rare benign neoplastic lesion that represents less than 2% of all cysts and odontogenic tumors; it is usually found at the canine region and is associated with odontoma. Knowledge on the lesion characteristics, a detailed clinical exam, and complementary exams are essential to a differential diagnosis aiming to provide appropriate treatment for the patient, which currently consists of complete enucleation, with good prognosis. The 12-month follow-up shows an injury-free patient