A 13-year-old girl was hospitalized for fever, malaise, intractable diarrhea, and intermittent gastrointestinal hemorrhage. Despite aggressive antimicrobial and supportive treatment, she died with massive bleeding from the upper gastrointestinal tract. Autopsy study revealed systemic polyarteritis nodosa of classic form in the right lung and gastrointestinal tract and of microscopic form in kidneys.