Although the pathogenesis of autoimmune pancreatitis is unclear, recent evidence of clinical aspects are presented: (i) mild abdominal symptoms, usually without acute attacks of pancreatitis; (ii) occasional existence of obstructive jaundice; (iii) increased levels of serum gammaglobulin, IgG or IgG4; (iv) presence of autoantibodies; (v) diffuse enlargement of the pancreas; (vi) irregularly narrowing of the pancreatic duct (sclerosing pancreatitis) with often intra-pancreatic biliary stenosis or coexistence of biliary lesions (sclerosing cholangitis similar to primary sclerosing cholangitis: PSC) on endoscopic retrograde cholangiopancreatographic (ERCP) images; (vii) fibrotic changes with lymphocyte and IgG4-positive plasmacyte infiltration, and obliterative thrombo-phlebitis; (viii) occasional association with other systemic lesions such as sialoadenitis, retroperitoneal fibrosis, interstitial renal tubular disorders, and (ix) effective steroid therapy. In addition to pancreatic and extra-pancreatic lesions, diabetes mellitus is occasionally responsive to steroid therapy. Further studies are needed to clarify the pathogenesis.