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Langerhans cell histiocytosis (LCH) is a rare condition most commonly reported in children aged less than 5 years. Paediatric dentists may be the first point of contact for this condition as it often manifests through the oral cavity and poses diagnostic challenges. The aim of this study was to report a case of a girl in her early childhood who had pain and tooth mobility in the upper and lower regions of the jaw. Intra-oral findings resembled those of aggressive periodontitis, while a routine pantogram and a few blood investigations suggested otherwise. A systemic evaluation was planned in collaboration with the Department of Paediatrics and Pathology, and LCH was the confirmatory diagnosis. Along with this study, a review of the literature was included in order to highlight the disguised presentation of LCH to a dentist and its impact on the child’s developing periodontium.