Abstract: Objective To describe the symptom of a man diagnosed with mixed-cellularity Hodgkin lymphoma (HL) complicated with hemophagocytic lymphohistiocytosis(HLH). Methods The patient presented with high fever, pancytopenia and jaundice. His fever was completely unresponsive to broad-spectrum antibiotics and then he was given steroid and ABVD (doxorubicin, bleomycin, vinblastine, dacarbazine) treatment. After treatment, his temperature was controlled temporarily while then raised high again with pancytopenia and deterioration of liver function. After a series of laboratory examination, he was diagnosed as HLH and given glucocorticoid. Results The disease was controlled temporarily and the related indexes were back to normal. Conclusion The present case is very rare with poor prognosis. Large scale studies are warranted to confrm the optimal treatment strategies.