An acute pancreatitis was observed in 2 children belonging to two unrelated families in which a total of 8 kindreds also had pancreatitis. Evolution was so severe that it demanded parenteral feeding and partial pancreatectomy with Wirsung-intestinal shunting. The authors stress: the rarity of this condition (which has a dominant autosomal transmission), the lack of specificity, the severity in which pseudocysts predominate, the further occurrence of both diabetes and external pancreatic deficiency and finally, the risk of pancreatic carcinoma.