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Langerhans cell histiocytosis (LCH) is a bone marrow-derived immature myeloid dendritic cell proliferative disorder with diverse clinical manifestations commonly involves bone, skin, lymph node and lung. Oral involvement is uncommon. Intraoral lesions can be the first sign of either a localized LCH or clinically undiagnosed systemic LCH, predates systemic manifestations of LCH, or an early indicator of recurrence in known cases. Clinically, it can be mistaken for primary oral and dental inflammatory, infectious and neoplastic lesions. H