Langerhans cell histiocytosis (LCH) is a rare clonal proliferative disorder that belongs to class I histiocytoses and is characterized by infiltration of one or many organs by Langerhans cells to form granulomas. The literature analysis could identify a lot of etiological, pathogenetic, and trigger factors and mechanisms for LCH development, which determine the diversity of the clinical picture and course of the disease. The clinical manifestations of LCH are very variable and depend on the severity of lesions and the age of patients. In addition to skin lesions, there is involvement of one or more visceral organs. Difficulties in diagnosing the disease lead to statistical variations of LCH in different countries of the world and require more attention by physicians of all specialties.