Autoimmune pancreatitis is a clinical entity with many different clinical and biological characteristics that make diagnosis difficult. Sensitive and specific pancreatic antibodies are still lacking to assess the diagnosis as is the availability of interpretable pathological specimens. However, suggestive features consist mainly of radiological findings and clinical anomalies, particularly if there are associated autoimmune-related diseases. The immunological pathway is probably of cell-mediated origin, although various autoantibodies, insensitive and non-specific, may exist. Finally, many studies are needed to define more efficient diagnostic criteria and to discover the true prevalence of autoimmune pancreatitis.