Leishmaniasis is a vector borne zoonotic parasitic disease transmitted by sand flies and it can be seen in all continents except Antarctica. It is a common disease in the countries located in Mediterranean basin (Turkey, Syria, North Africa, Jordan, Italy, Iran). It may present with three clinical forms such as cutaneous, mucocutaneous visceral leishmaniasis (VL). VL is an endemic disease in the Mediterranean and Aegean regions of Turkey and it is seen sporadically in the other regions. In this report a case who is living in Izmir province with hemophagocytic lymphohistiocytosis (HLH) secondary to VL was presented. A 50-year-old woman admitted to our department with fever, weakness, sweating and fatique for about three weeks. The patient's temperature was 38.7 degrees C. Laboratory tests revealed pancytopenia with hemoglobin level of 8.91 g/dl (11.7-16.0 g/dl), platelet level 10(4) x 10(3)/mu l (150-450 x 10(3)/mu l) and leukocyte (1.35 x 10(3)/mu l (4.5-11 x 10(3)/mu l) counts. Ferritin and triglyceride levels were increased. Ferritin level was 764.4 ng/ml (13-150 x 10(3)/mu l) and triglyceride level was 232 mg/dl (< 150 mg/dl). HLH was diagnosed according to the 2004 HLH current diagnostic guidelines. After this diagnosis the patient was investigated for underlying disease such as malignancy, autoimmune diseases, medications and infectious diseases. Bone marrow aspiration showed hemophagocytosis and intracellular Leishmania amastigotes. Leishmania indirect fluorescent antibody IgG (IFAT) was positive in titre of (1/1024). In addition, in the bone marrow aspiration sample taken from the patient, the causative agent of leishmaniasis was searched by polymerase chain reaction, and it was determined that etiological agent was Leishmania infantum. The patient was evaluated as HLH secondary to VL. Liposomal amphotericin B therapy was started and in the 5th day of treatment, the patient's fever was measured as normal. There was a significant decrease in fatigue and sweating. After two months of treatment, the complaints disappeared completely and hemoglobin, leukocyte, platelet counts were found normal. HLH and VL can be fatal if they are not early diagnosed and treated. Physicians must be alert for HLH when cytopenia, uncontrolled fever, high triglyceride and high ferritin level and parasitological tests should be done for possible underlying disease. In this case report, we aimed to draw attention to the diagnosis of HLH, which is a very rare clinical presentation of leishmaniasis.