Langerhans cell histiocytosis (LCH) is a rare disease characterized by abnormal accumulation of immature dendritic cells in tissues. With the discovery of BRAFV600E gene mutation, the etiology and pathogenesis of LCH have become clear, but the diverse clinical manifestations of LCH still result in difficulties of its treatment. This article reviews the latest progress in the etiology, pathogenesis and treatment of LCH in recent years.