Abstract: Objective　To explore the clinical features of haemophagocytic syndrome secondary to tsutsugamushi disease in children. Method　The clinical data of a child with hemophagocytic syndrome secondary to tsutsugamushi disease were retrospectively analyzed. Domestic and foreign periodical databases were searched, and the clinical data of hemophagocytic syndrome secondary to tsutsugamushi disease in 32 children were collected and analyzed. Results　A 9-month-old girl was admitted due to fever of unknown origin for 9 days with an episode of seizure. She was diagnosed of hemophagocytic syndrome secondary to tsutsugamushi disease 3 days after admission because 5 clinical symptoms including fever, splenomegaly, hematocytopenia, serum ferritin elevation, hypofibrinogenemia or hyperlipemia were among the HLH-2004 diagnosis criteria (8 items), together with its characteristic eschar and positive antibodies of scrub typhus. The body temperature dropped to normal 3 days after anti-infection treatment with azithromycin and all the indicators were gradually improved. In the search, a total of 32 patients (excluding this child) with hemophagocytic syndrome secondary to tsutsugamushi disease were retrieved, all of whom were Asians, 16 males and 16 females, aged from 2 months to 11 years. Eschar was found in 24 cases, splenomegaly in 30 cases, and decreased serum albumin in 30 cases. Decreased blood cells and elevated serum ferritin levels were observed in almost all of the children. Thirty-one children were misdiagnosed at first. Twenty-seven children were treated with appropriate antibiotics, 16 with a combination of gamma globulin, 21 with corticosteroids, and only 1 with etoposide chemotherapy. Most children no longer had a fever after treatment from 24 hours to 4 days, and 5 died. Conclusion　The hemophagocytic syndrome secondary to tsutsugamushi disease is often accompanied by a decrease in albumin and requires appropriate antibiotic and immunomodulatory treatment, and most of the patients have good prognosis.