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A 73-year-old woman with erythematous nodules was admitted to our hospital in December 2003. She was diagnosed with polyarteritis nodosa (PAN) from skin biopsy and laboratory data. Following the treatment with oral prednisolone (40 mg/day), her condition improved. Four days after the reduction of prednisolone, she became febrile. Bone marrow aspiration revealed an increase in the number of marrow macrophages, and phagocytosis of blood cells. The Epstein–Barr virus genome was detected in her peripheral blood. A diagnosis of hemophagocytic syndrome was made. Moreover, intestinal bleeding developed and the patient was given medical treatment consisting of methylprednisolone pulse therapy, intravenous immunoglobuline, weekly intravenous VP-16, and several blood transfusions. In addition, embolization of a branch of the ileal artery was performed. Despite the above treatments, the patient died. Autopsy revealed hemophagocytosis in bone marrow and perforation of ileocecal region. This case suggests that risks for hemophagocytic syndrome in PAN patients should be recognized. [ABSTRACT FROM AUTHOR]