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.