The association of hemophagocytic lymphohistiocytosis (HLH) with Hodgkin disease (HD) seems to be uncommon. A 9-year-old boy, diagnosed with HD-HLH, received chemotherapy and remained in complete remission for 21 months. Then the patient received the first autologous transplant and unfortunately died due to multiorgan failure after the second allogeneic transplant because of sequentially respective relapse of HD and HLH. The recurrence of HLH with a high Epstein-Barr virus load but without relapse of HD in the patient suggests that Epstein-Barr virus may have a major pathogenetic role in the specific disorder HLH-HD. Detailed reports relating to HLH-HD were also reviewed.