Autoimmune pancreatitis (ALP) represents a distinct form of chronic pancreatitis initially described in Japan but now reported worldwide. AIP often presents with obstructive jaundice/pancreatic mass as well as pancreatic exocrine and endocrine insufficiency. Histologically, it is characterised by a lymphoplasmacytic infiltrate with fibrosis. The disease responds readily to steroids in 70-80% of cases. Given the absence of unified diagnostic criteria, the diagnosis of AIP proves difficult. In particular, distinguishing ALP from pancreatic or biliary cancer remains a challenging task. In order to avoid unnecessary resections for an otherwise benign and easily treatable condition, it is urgent to refine diagnostic criteria and to reach an international consensus.