Lymphocyte-rich classical Hodgkin lymphoma (LRCHL) is a rare subtype of Hodgkin lymphoma with a favorable prognosis, and an aggressive clinical course of LRCHL is uncommon. A 55-year-old man suffering from swelling in the left neck was diagnosed with LRCHL with extranodal lesions in the lung and bone marrow. Initially, he received standard ABVD chemotherapy; however, disease progression, accompanied by hemophagocytic syndrome (HPS), occurred during the second course of ABVD. He received two subsequent courses of intensive chemotherapy containing high-dose steroids, cyclophosphamide, and etoposide. Nevertheless, this therapy was only temporarily effective, and he died of due to an aggressive disease progression accompanied by uncontrollable HPS and severe coagulopathy.