The cause of the pseudocyst was chronic pancreatitis in two children (associated with hypermobility-type Ehlers–Danlos syndrome in one child), acute pancreatitis in two children (10 weeks following heart transplant in one and following commencement of asparaginase for acute lymphoblastic leukaemia in another), recurrent pancreatitis in one child secondary to a serine protease 1 (PRSS1) mutation, and blunt-traumatic transection of the pancreas in one child. Here, PRSS1 is linked to acute pancreatitis.