Hepatosplenic T-cell lymphoma (HTCL) is a rare malignancy. Prognosis is poor with only a few case reports of long-term survivors. While HTCL universally involves the bone marrow, the condition has been most often treated with multimodal lymphoma specific chemotherapy. We report a durable, sustained first remission in an adolescent treated for HTCL who received induction therapy according to a high risk T-cell leukemia regimen, a nucleoside analog-based consolidation, and allogeneic transplantation associated with GVHD.