The success of endodontic therapy is based on having sufficient endodontic access, correct cleaning and shaping, and adequate root canal obturation. However, endodontic treatment is also dependent on having a sound knowledge of the internal anatomy of human teeth, especially when anatomic variations are present. Reporting these alterations is important for improving the understanding and expertise of endodontists. The aim of this case report is to describe a unique case of maxillary first molar with 2 palatal canals within a single root, as confirmed by cone-beam computed tomography (CBCT) scans. This article also reviews recent case reports of extra palatal root canals in the maxillary first molars and the role of CBCT analysis in successfully diagnosing them.