Autoimmune pancreatitis is becoming a more widely recognized form of pancreatitis that can mimic pancreatic or biliary malignancy. The combination of serological, histological and radiographic findings makes it unique among pancreatic diseases. The presence of autoantibodies, IgG4 and a lymphoplasmacytic infiltrate reflect its autoimmune etiology. The dramatic response to steroids is also a distinguishing feature and differentiates it from other pancreatic diseases.