Abstract: Objective：To evaluate the efficacy of allogeneic hematopoietic stem cell transplantation (allo-HSCT) in the treatment of children with hyper-IgE syndrome （HIES）caused by DOCK8 gene mutation. Methods：Clinical features, laboratory data, treatment and outcome of 2 patients with HIES caused by DOCK8 gene mutation treated by allo-HSCT were retrospectively analyzed. Results：There was a boy and a girl in 2 cases with HIES. The age of allo-HSCT was 7 for the boy and 9 for the girl. The boy received matched sibling donor allo-HSCT, and the girl received matched unrelated umbilical cord blood transplantation. Reduced-intensity conditioning regimens based on Busulfan and Fludarabine were used for the 2 patients. Both patients were successfully engrafted and achieved hematopoietic reconstruction. After allo-HSCT, neutrophil engraftment occurred on day 12 and 24, and platelet engraftment occured on day 13 and 35 for the boy and girl, respectively. Both patients showed stable complete donor chimerism 14 days after allo-HSCT. Pulmonary infection in both patients aggravated during transplantation, and conditions were improved with active therapy. The boy who received matched sibling donor allo-HSCT suffered grade Ⅰ acute skin graft-versus-host disease (GVHD), and his condition was alleviated after the methylprednisone treatment. No other complications were reported. The levels of eosinophils and serum IgE were much lower than those before transplantation. Two children survived disease-free for 18 months and 23 months after transplantation. Conclusion：Children with HIES caused by DOCK8 gene mutation can be cured by allo-HSCT. It is advisable to adopt a reduced-intensity conditioning regimen. Matched sibling donors are the best choice for allo-HSCT, and unrelated umbilical cord blood stem cell transplantation can also achieve good results.