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Variation in the root and canal morphology of maxillary second molars is quite common. The most common configuration is three roots and three or four canals. But the presence of an additional palatal root is rather uncommon and has been reported to have an incidence of 1.4%. The extra root is located mesiolingual or distolingual to the primary palatal root. This is a unique case report that describes the successful endodontic management of a maxillary second molar with Radix Distolingualis (RDL). Identification of this variation was done through clinical examination, multi-angled radiographs, surgical operating microscope and an accurate assessment of this morphology was made with the help of Cone Beam Computed Tomography (CBCT) imaging. In addition, this article also discusses the literature review, guidelines for diagnosis and treatment of RDL.