Background: Hereditary Pancreatitis is a rare cause of acute pancreatitis. These patients usually present at a young age with recurrent episodes of acute pancreatitis that usually progresses to chronic pancreatitis with associated endocrine and exocrine failure. Hereditary pancreatitis also carries an increased lifetime risk of pancreatic adenocarcinoma. These high risk patients need early intervention where appropriate by specialist multidisciplinary teams and tailored long term management. Methods: A review of the relevant literature regarding the epidemiology, the genetic mechanisms responsible for the disease and current management strategies for Hereditary Pancreatitis was performed. Results: Several underlying genetic driver mutations for Hereditary Pancreatitis have been identified, notably PRSS1, SPINK1, CFTR and CRTC mutations. Recurrent episodes of pancreatitis result in a progressive loss of normal pancreatic parenchyma, which is