Systemic EBV positive T-cell lymphoma of childhood (S-EBV-TCL) is a rare disease for which there is no standard of care. S-EBV-TCL is often associated with hemophagocytic lymphohistiocytosis (HLH) and is generally thought of on the spectrum of Epstein-Barr Virus (EBV) related disease. For the few reported cases of cure in the literature, hematopoietic stem cell transplant (HSCT) has been required. Here, we present the case of one patient who was successfully cured with a modified regimen of dose adjusted EPOCH (etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin), followed by HSCT using a reduced intensity conditioning regimen.