Hereditary pancreatitis presenting with ascites

We report a case of an adolescent girl who presented with painless massive ascites secondary to chronic pancreatitis and a ductal fistula. The diagnosis was delayed and an unnecessary laparotomy was performed, as initial evaluation of ascites did not include measurement of serum and ascitic amylase. Evidence of pancreatic abnormalities in asymptomatic relatives suggested an underlying hereditary pancreatitis. Hereditary pancreatitis presenting as pancreatic ascites, to our knowledge, has not been described previously.