We report a case of a 7-year-old Chinese boy who presented with acute fever, multiple oral ulcers, and skin nodules. A diagnosis of systemic Epstein-Barr virus (EBV)-positive T-cell lymphoma of childhood was established using systemic laboratory examination, imaging studies, bone marrow and skin biopsy with immunohistochemistry, and in situ hybridization for EBV-encoded RNA (EBER) and gene rearrangements. Notable features of this case include the absence of pancytopenia and hemophagocytic syndrome as well as spontaneous resolution without chemotherapy for several months; however, the condition relapsed, and the patient died.