Objective: To investigate the clinicopathological characteristics of systemic Epstein-Barr virus (EBV) positive T-cell lymphoma of childhood and improve the understanding of the new diagnostic criteria. Methods: Clinical presentations, pathological characteristics, gene detection results and immunohistochemical phenotyping of biopsy tissue from a case of systemic EBV positive T-cell lymphoma of childhood were analyzed and the relevant literature was reviewed. Result: The 11-year-old boy with prolonged mite allergy treatment and no responsive rash on limbs and torso presented recurrent high fever, generalized lymphadenopathy and hemophagocytic syndrome. Lymph node biopsy revealed diffuse proliferation of small to intermediate neoplastic T cells, which was possibly originated in interfollicular area and positive for CD3（+++）, CD4（+）, TIA-1（+++）, GranzymeB（+++） and MIB-1 (>80% positive). The in situ hybridization of EBV (EBER) was positive （+++） and clonal TCRγ gene rearrangemen was also detected. The diagnosis of systemic EBV positive T-cell lymphoma was thus established. Conclusions: Systemic EBV-positive T-cell lymphoma of childhood is a rare systemic illness characterized by clonal proliferation of EBV-infected T cells and aggressive and fulminant clinical course often associated with hemophagocytic syndrome, which requires differentiation with medical conditions such as invasive NK cells, leukemia/lymphoma, EBV infection associated lymphoproliferative lesions and primary cutaneous gamma-delta T-cell lymphoma.