Adult-onset Henoch-Schönlein purpura (HSP) is a rare systemic vasculitis characterized by a leukocytoclastic vasculitis of small vessels with the deposition of IgA immune complexes involving skin, gastrointestinal tract, joints and kidneys. Antineutrophil cytoplasmic antibody (ANCA) detected by indirect immunofluorescence assay is commonly found in other vasculitic disorders but rarely discovered in HSP patients. ANCA with perinuclear pattern has hardly ever reported in HSP patients. The diagnostic importance of ANCA still remains controversial. In addition, the simultaneous presence of diabetic nephropathy and HSP is uncommon. We present a case of an adult patient with diabetic nephropathy and superimposed HSP, which resulted in acute renal failure. Perinuclear-pattern ANCA was detected in the acute phase of HSP but disappeared when the disease resolved. Further, we have reviewed ANCA-positive HSP in this article.