Langerhans Cell Histiocytosis is a rare non malignant disease characterized by a clonal proliferation of pathologic cells with the characteristic Langerhans cells in single or multiple sites and an unpredictable course. The clinical presentation is heterogenous ranging from single system involvement to a multisystem life threatening disease. We report a case of LCH in 2 yr old female child with clinical signs of portal hypertension and hepatic involvement.

Keywords: Langerhans Cell Histiocytosis; Portal Hypertension; Hemetemesis.