Immunoglobulin G (IgG) 4-related disease (IgG4-RD) is a newly recognized fibroinflammatory entity characterized by elevated serum levels of IgG4 and histological infiltration of IgG4+ plasma cells. Several inflammatory diseases like autoimmune pancreatitis and Mikulicz’s disease were previously regarded as independent, organ-specific diseases but they are now unified as IgG4-RD in terms of IgG4. However, since there have been reported that Anti-neutrophil cytoplasmic antibodies (ANCA) has close connection with an increased IgG4 immune response, IgG4-RD may overlap with ANCA-associated vasculitis. A 46-year-old man presented with violaceous papules on both legs for 3 days. He complaint for fever and cough over 3 weeks and followed by polyarthralgia and petechia. C-ANCA was detected and serum IgG4 levels were elevated on laboratory examination. Skin biopsy specimen from his right leg showed features of leukocytoclastic vasculitis with perivascular granulomatous lymphoplasmacytic infiltration. Chest X-ray revealed pneumonic infiltration in the left lower lobe and percutaneous lung biopsy was performed. From the lung biopsy specimen, diffuse fibrosis with heavy lymphoplasmacytic infiltrates were observed and abundant IgG4+ cells were identified. From the laboratory and histologic findings, he was diagnosed with IgG4-RD overlapped with ANCA-associated vasculitis.