RESUMO
Systemic polyarteritis nodosa (PAN) is a rare disease in childhood affecting small and midsized arteries. The typical presentation in children is of isolated 1- or 2-organ involvement, and the diagnosis is often based on the histopathology. We report a case of pediatric PAN whose presenting symptom was jejunal obstruction owing to ischemic necrosis of the small bowel. The postoperative recovery was uneventful, and the patient responded well to steroid therapy. There are only a few cases of PAN with intestinal involvement described in the literature, some of them fatal. The diagnosis of PAN should be considered in children with acute intestinal obstruction with no obvious etiology, as early recognition and treatment may reduce its high mortality.