A 20-year-old Caucasian man presented to our clinical Department for the correction of an excessively wide maxillary tooth and an anterior diastema that caused aesthetic and psychological problems. His medical history was noncontributory and there were no dental abnormalities among his family members. An oral investigation revealed the dental anomaly and a missing tooth with regard to normal dentition. A diagnosis of fusion of two central incisors was made according to the definition of Braham []. We also found a right central incisor dyschromia, a diastema between the fused teeth and lateral left incisor, and a dental misalignment. The fused teeth crown showed an evident palatal and buccal groove, extending 2mm subgingivally. Vitality pulp tests were negative for central incisors and right lateral incisor. A radiographic investigation showed a fused tooth with separate pulp chambers, two distinct roots and two separate root canals associated with periapical lesions of central incisors and right lateral incisor. A presumptive diagnosis of a radicular inflammatory cyst was made. The treatment plan called for endodontic treatment, teeth separation followed by orthodontics, and prosthetic rehabilitation. Because of the teeth necrosis and presence of a periapical lesion, conventional endodontic treatment was performed. His tooth was isolated with a rubber dam, an access cavity was prepared on the medial and distal part of his tooth and the pulp was extirpated. No communication was detected between the two pulp chambers using a curved probe. The root canals were cleaned and shaped, temporized with calcium hydroxide, and sealed. One week later, the endodontic treatment was completed. After six months, a radiographic control revealed the persistence of periapical radiolucency. We then decided to perform endodontic surgery, which included exeresis of the lesions, apicoectomy, and retrograde obturation with a reinforced zinc oxide-eugenol cement (SuperEBA). Complete healing of the lesion was obtained six months postoperatively and orthodontic treatment was initiated after an evaluation of his molar class, overbite, overjet and so on. After one week, orthodontic appliances were put in place. At the same appointment, buccal and palatal flap were raised and the fused teeth crowns were separated along the buccal groove with a diamond bur. Because of the presence of an anomalous labial frenulum, a frenulectomy was indicated. Nine months later, the correct position of teeth was obtained and the anterior teeth were prepared to receive crowns. The provisional crowns were tested for two weeks before fabrication of the definitive prostheses to achieve the proper maturation of soft tissue. The definitive prostheses were made in ceramic material. The final aesthetic result was acceptable and our patient was satisfied. A postoperative radiograph performed one year later showed no signs of periapical pathologies.