Abstract:  Objectives To analyze the clinical features and outcome of typhoid fever complicated with hemophagocytic syndrome (Ty-AHS) in children. Methods The clinical data from one case of Ty-AHS was retrospectively analyzed, and related articles were reviewed. Results A 4-year-old boy suffered from persistent diarrhea, alternating high and low temperature, apathia, hepatosplenomegaly, manifestation of acute peritonitis, and pyoperitoneum. Routine blood examination showed that eosinophil was 0, and hemoglobin and platelet were obviously decreased; CRP and procalcitonin were obviously increased; plasma fibrinogen was dropped to 0.8 g/L; lactate dehydrogenase was elevated to 3835 U/L; Serum ferritin was 1884 ng/mL; triglyceride was 2.42 mmol/L; EBV-DNA titer was 2.81×104 copies/mL; Blood culture showed salmonella enterica serotype IIIb. Abdominal ultrasonography showed enlargement of mesenteric lymph node and middle volume of pyoperitoneum. Chest X-ray showed pneumonia. Lymphocyte analysis showed that the ratio of CD4+/CD8+ was decreased; CD3－CD16+56+ cell and CD19+ cell were all decreased. Bone marrow cytomorphologic examination revealed that bone marrow hyperplasia was active, there were no obvious abnormalities in granulocyte, macrophage and macrophage and there were a lot of tissue cells and white blood cells. After two weeks of strengthened anti-infection and dexamethasone treatment, the symptoms in patients were disappeared, and signs and laboratory tests gradually returned to normal. Conclusions Ty-AHS is a rare complication in children with acute onset and rapid progression, and combination of antibiotics and hormone therapy is effective.