Autoimmune pancreatitis is a rare condition characterized by inflammation and scarring of the pancreas caused by an autoimmune process. It has been referred to by numerous names since its initial description in 1961. A national survey in Japan found a prevalence of 0.82 cases per 100,000 people. Clinical presentations include acute recurrent pancreatitis, painless obstructive jaundice that must be differentiated from pancreatic cancer, and subacute or late presentations involving a persistent pancreatic mass, an atrophic pancreas with possible calcifications, or unexplained steatorrhea.