Atypical anatomy in the maxillary incisor is extremely rare, with only a few case reports describing these variations. Cone-beam computed tomography (CBCT) is an effective way to accurately diagnose anatomical disparities and determine the 3-dimensional location of roots and canals. The aim of this article is to report on the diagnosis and endodontic treatment of a rare case of a double-rooted maxillary central incisor, supported by CBCT scanning as a diagnostic imaging technique. Clinical examination of a 72-year-old patient who was referred for endodontic treatment of tooth No. 9 revealed a coronary fracture and no response to thermal sensitivity tests. Based on the periapical radiographs, the presence of anatomical variations was suspected. This prompted a CBCT scan for the acquisition of a more accurate diagnosis, and two roots were detected. This article details the successful endodontic treatment performed. The results demonstrate that in a case of abnormal anatomy in a maxillary incisor an effective diagnosis along with adequate treatment may allow for a favorable prognosis in the long term.