Childhood polyarteritis nodosa ( PAN) is a rare systemic inflammatory disease characterized by systemic necrotizing vasculitis of the small or medium sized arteries that leads to infarct, and aneurysms in various organs. A 7 year-old girl was admitted due to sudden onset of painful blackish discoloration of right 2nd, 4th and left 2nd finger tips from proximal interphalangeal joints with fever. She could not walk by herself because of myalgia on both lower extremities. On admission, body temperature was 38.5℃, and blood pressure 150/86 mmHg. Physical examination showed gangrenous change of finger tips and tender swelling on right ankle joints. Initial laboratory data showed leukocytosis with dominant neutrophils, high C-reactive protein and ESR. Autoantibodies including ANA and ANCA showed negative. We identified microscopic hematuria. CT angiography for upper and lower extremities showed diffuse swelling with soft tissue fatty infiltration on both fingers, but no definite vessel and its branch abnormalities and renal arteries. Brain MRA showed luminal irregularity at intracranial major arteries compatible with arteritis, but no definite aneurysm. Electromyographic findings for motor showed decreased amplitudes in right median and ulnar nerves. Skin biopsy showed perivascular lymphohistiocytic infiltration compatible with vasculitis without any immunofluorescent staining. Culture for skin, blood, and urine was no any organisms. We diagnosed with systemic PAN including skin change, arthralgia and myalgia, hematuria, histologic findings and neuropathy. We started with low molecular weight heparin, prostaglandin E for prevention of thrombisis, and morphin for pain control. She received intravenous immunoglobulin and steroid. She was improved symptoms after steroid therapy, but still no changes of digital gangrene. We changed anti-coagulation therapy to warfarin. She was tolerable after oral steroid and warfarin. Now, she was tolerable and follow up at out patient clinic in spite of self amputation of finger tips. We present herein a 7-year-old girl with systemic PAN including digital gangrene, arthralgia, myalgia, microscopic hematuria and neuropathy.