Langerhans Cell Histiocytosis (LCH) is a rare granulomatous disease with an unknown origin. LCH occurs at any age and affects any organ. It is presented as self-limited to aggressive forms. Late diagnosis of LCH, after the evidence is revealed at the radiological imaging or microscopic investigations, aggravates the possible complications. This study reported a rare case of LCH with a bone lytic lesion at the right scapula with a good prognosis.