This paper describes a 5-year-old Chinese girl who presented with acute fever and epistaxis. After admission, relevant ancillary tests indicated the presence of hemophagocytic lymphohistocytosis (HLH) and the combination of EBV infection in this patient. Pathology of the cervical lymph node biopsy and bone marrow flow cytology examination indicated systemic EBV-positive T-cell lymphoma of childhood (STCLC), and a diagnosis of STCLC combined with HLH was clear. Although the girl was clearly diagnosed within a few days and treated with chemotherapy and symptomatic support, she eventually died on the 6th day after admission due to progressive worsening of her disease. We also describe the possible pathogenesis of STCLC combined with HLH through a search of the relevant literature.