Langerhans cell histiocytosis (LCH) is a disease involving formation of granulomatous infiltrations consisting of Langerhans cells, histiocytes, lymphocytes and eosinophilic granulocytes. Etiopathogenesis of this disease has not been fully clarified yet. It can present as a focal and disseminated form (acute and chronic). Rare incidence of this disease in the population causes progress in research and new therapeutic methods to be slow. Basing upon a case of a patient treated for gingivo-osseous lesions in the course of LCH, we tried to prove that a dental or maxillofacial surgeon may be the first contact medical professional who contributes to the diagnosis of LCH as a systemic disease.