Polyarteritis nodosa (PAN) is a rare vasculitis in children. We report a case of 5-year-old boy with systemic PAN and cholestatic hepatitis. He had fever, abdominal pain, and gastrointestinal bleeding. Angiography revealed multiple microaneurysms in the renal, hepatic, and superior mesenteric arteries. Clinical manifestations improved slowly after immunosuppressive therapy, but liver enzyme and bilirubin levels elevated gradually. Liver biopsy findings revealed marked centrizonal canalicular cholestasis, bile duct damage, and intact hepatocyte, but there was no evidence of viral hepatitis or vasculitis. Levels of liver enzymes and bilirubin improved after two cycles of cyclophosphamide therapy. We thought that the possible etiology of elevated liver enzyme and bilirubin levels might be a manifestation of PAN.