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Langerhan’s Cell Histiocytosis (LCH) is disorders which include abnormalities that result from abnormal proliferation of langerhan’s cells or their precursors. LCH is clinically classified into three types-eosinophilic granuloma, Hand Schuller Christian disease and Abt-LettererSiwe disease. It is usually manifested in childhood as well as in adulthood. The clinical manifestations are the result of the accumulation and infilteration of the langerhan cells in organs and tissues. Here is a rare case report of 6-year-old boy with extraoral manifestation of exopthalmic right eye and oral manifestation of mobility of teeth and with typical radiological findings. Basing on the clinical, radiological and histopathological examination the diagnosis of Hand Schuller Christian Disease was given.