Background: Hemophagocytic lymphohistiocytosis (HLH) frequently is a fatal disorder of immune system, mainly manifesting as uninterrupted fever, hepatosplenomegaly, pancytopenia, hemophagocytosis, central nervous system (CNS) involvement, and elevation of HLH related biomarkers. Hematopoietic stem cell transplantation (HSCT) is the sole curative therapy for this disease. Methods: Retrospective study of outcomes in patients who underwent HSCT in a single center. Results: Eight patients (four males), with a median age at diagnosis was 3.3 years (range, 1.2 to 13.4) and at transplantation was 2.9 years (range, 0.8 to 13.0), received standard HLH chemotherapy before HSCT. Two patients were in active disease (AD). All received busulfan-based myeloablative conditioning (MAC) regimen. Four (50%) patients received peripheral blood stem cells (PBSC), four (50%) received umbilical cord blood (UCB). Two (25%) patients who received UCB first underwent second transplantation. Three (37.5%) patients had acute Graft-versus-host disease (GVHD), and one (12.5%) had chronic GVHD. Only one (12.5%) patient died due to multiple organ dysfunction syndrome (MODS). Conclusion: Active disease and mixed chimerism were the fatal prognostic factors. Graft failure was a part of the significant disadvantages of UCB. In combination with standard HLH therapy, HSCT could be an effective means of prolonging survival and curing pediatric HLH. To some extent, UCB is a good option in the absence of PBSC.