Objective To analyze the clinical data of children with visceral leishmaniasis (VL) presenting as hemophagocytic syndrome (HPS) in order to improve the clinical diagnosis and treatment level. Methods The clinical data of 4 children with HPS secondary to VL hospitalized from March 2020 to March 2021 were retrospectively analyzed, and were compared with the literature data. Results All the 4 patients showed persistent fever, splenomegaly and cytopenia. Combined with relevant examinations, the patients were diagnosed with HPS. Considering the epidemiological factors, the examination of VL was performed, and the results showed that rK39 test was positive in 4 cases, and Leishmanias donovani was detected by bone marrow smear in 3 cases. Sodium stibogluconate treatment in the four children was successful. A review of the literature on VL-HLH in the past 5 years showed that compared with primary HLH, children with VL-HLH had higher ferritin and increased erythrocyte sedimentation rate. Compared with VL, VL-HLH may be more prone to pancytopenia, hypertriglyceridemia, hyperserum ferriminemia, and lower absolute monocyte counts. Amphotericin B may be more efficient and safer than sodium stibogluconate for the treatment of VL-HLH.Conclusions For children with a history of staying in the epidemic area, the possibility of VL should be considered in the comprehensive analysis of the primary etiology of HPS, and rK39 test and bone marrow smear examination should be performed at the same time. Early and accurate treatment of the cause can avoid aggravation of the disease and the toxic side effects caused by chemotherapy.