Polyarteritis nodosa (PAN) is a rare, systemic necrotizing vasculitis of medium-sized arteries. The American College of Rheumatology criteria for the diagnosis of PAN includes at least three of: 1. weight loss < 4 kg, 2. livedo reticularis, 3. testicular pain or tenderness, 4. myalgias, weakness or leg tenderness, 5. mono-or polyneuropathy, 6. diastolic hypertension, 7. elevated blood creatinine or urea, 8. hepatitis B antigen or antibody in the serum, 9. aneurysms or occlusions of visceral arteries or 10. granulocytes on small or medium sized artery biopsy. Common sites of involvement include skin, joints, kidneys, gastrointestinal tract and peripheral nerves. Central nervous system involvement has been reported in up to 40% of cases; the usual manifestations are encephalopathy, focal deficits and seizures. Descriptions of PAN in the pediatric literature has been reported in fewer than 250 children.