Langerhans cell histiocytosis (LCH) is a rare disease of unknown etiology characterized by oligoclonal proliferation of Langerhans cells. The diagnosis of LCH is complicated by the fact that it may involve multiple organ systems and its clinical presentation and course varies, ranging from an isolated to a multisystem disease. In children, the estimated incidence of LCH is eight to nine cases a million each year [1] and affects childrens most commonly 1-5 yrs of age group. we report here an unusual case of a one n half -year-old female diagnosed with Langerhans cell histiocytosis initially taking course under the form of leukemia with bleeding gums ,anemia and generalized peripheral lymphadenopathy, also presented with icterus, anasarca and raised liver enzymes, subsequently leaving the clinicians in a dilemma to arrive at a correct diagnosis .The earliest diagnosis was then determined by doing FNAC of lymph node , following which skin biopsies and IHC were done which confirmed the case to be LCH.
