There are genetic mutations taking part in the physiopathology of pancreatitis. The role of alpha-1-antitrypsin (AAT) deficiency in this pathology is debated. We report the case of a 60-year-old man with a pancreatic exocrine insufficiency. He was diagnosed with AAT deficiency. The phenotype was Pi SZ, with genotyping confirmation. The place of AAT deficiency in the midst of pancreatic diseases should be further studied.