A rare case of polyangiitis overlap syndrome is described. The patient was a 25-year-old man who had palpable purpura on his legs which showed leukocytoclastic vasculitis, and polyarteritis nodosa. Superior mesenteric arteriography showed microaneurysms in jejunal branches with focal segmental necrotizing arteritis of small and medium sized muscular arteries in the jejunum. Deposits of IgA and C3 in the superficial blood vessels of the lesional skin were consistent with the features of Henoch-Schönlein purpura. The patient died about two months after initial admission in spite of cytotoxic agent and steroid administration.