In 1961, Sarles et al. first reported a case of chronic pancreatitis (CP) with hypergammaglobulinemia. Autoimmune pancreatitis (AIP) is the form of CP with still unclear pathogenesis. Clinical diagnosis of AIP is established o the basis of characteristic laboratory signs (increased levels of serum gammaglobulin, especially lgG4, presence of autoantibodies), typical changes observed in diagnostic image procedures (diffuse enlargement of the pancreas, irregular narrowing of the main pancreatic duet) and fibrotic changes with lymphocyte and lgG4-positive plasmocyte infiltration. The clinical course of AIP is often asymptomatic or with only mild symptoms, steroid therapy is effective.