There are two types of autoimmune pancreatitis. Type 1 is a part of a polyexocrinopathy syndrome associated with an elevation of serum igG4. Other organs might be involved: salivary glands, biliary ducts, retroperitoneum, kidneys, etc. Relapse risk is elevated and evolution to exocrine and endocrine insufficiencies is possible. Type 2 is not linked to 16gG4 and might be associated to inflammatory bowel diseases that may occur before, during or after pancreatitis. Relapse risk is lower than for type 1. Main clinical manifestations are jaundice (type 1) or acute pancreatitis (type 2). The diagnosis relies mainly on a fine analysis of ductal abnormalities on MRCP with thick slices. Pancreatic lesions might be diffuse, multifocal or pseudotumoral. In the latter case, there is a high risk of misdiagnosis leading to inappropriate pancreatic resection. In doubtful cases, a pancreatic biopsy is warranted. Indications for treatment rely on symptoms. Corticosteroids and rarely immunosuppressive therapy or rituximab are the basis of treatment.