We present an one year-old boy and a 4-month old girl from Iran with hemophagocytic lymphohistiocytosis (HLH) associated with Leishmania. HLH is not an independent disease but rather a life-threatening clinical syndrome that occurs in many underlying conditions and in all age groups [1] . It is commonly appears in infancy [2] . In our cases, treatment with Amphotericin B was started and first case responded and second case unfortunately died.