Background. Hemophagocytic syndrome (HPS) is characterized by a hyperinflammatory reaction followed by alteration in cytotoxic function of Th1 lymphocytes and natural killer cells. We report a rare case of a patient that presented with fever and pancytopenia due to HPS associated with Hodgkin's lymphoma (HL). Case Report. A 69-year-old Caucasian woman was admitted presenting with complaints of fever, seizures, and low back pain that had lasted for two weeks. Laboratorial data showed pancytopenia. Bone marrow biopsy revealed infiltration by Reed-Sternberg cells and hemophagocytosis signs. Imaging studies showed mediastinal lymph nodes (stage IV B). She had been treated with ABVD (doxorubicin, bleomycin, vinblastine, and dacarbazine) followed by a good response. Conclusion. HPS associated with HL is a very rare and lethal disease, with mortality rates of about 15% to 60%. The prompt diagnosis of the underlying lymphoma may be an important strategy for optimizing the clinical approach and outcome.