“…The authors conclude that there may be a relationship between KM and mucopolysaccharidosis, but impacted molar molars are not a pathognomonic factor, and RF may exist in patients without systemic alterations as an isolated characteristic. The mucopolysaccharidosis is inherited metabolic disorders, in which there will be abnormal deposition of mucopolysaccharides in the tissues like, skin, soft tissues of airway, cornea, central nervous system, heart, liver, spleen, bones, ligaments, and other sites due to an enzyme abnormality (Shahista, et al, 2013) The KM mainly involve second and third lower molars, with a higher prevalence of bilateral cases (n=9) (Van Hoof, 1973;Zerener, et al, 2016;Robinson, et al, 1991;Fortes, et al, 2014;Anish, et al, 2015;Ghosh, et al, 2017;Jannu, et al, 2014;Kiran, et al, 2014;Nakamura, et al, 1992;Nedjat-Shokouhi & Webb, 2014) followed by 7 cases involving teeth #47 and #48 (Gonzalez-Perez, et al, 2015;Arjona-Amo, et al, 2016;Scheuber & Bornstein, 2014) and 5 cases of teeth #37 and #38 (Fortes, et al, 2014;Ghosh, et al, 2017;McIntyre, 1997;Bozkurt, et al, 2017). In the literature, there is only 1 case of KM involving teeth #46 and #47, described by Barros, et al (2018) and 1 case involving third and fourth molar quarters,…”