Background: Hereditary pancreatitis (HP) is an uncommon condition resulting from an imbalance of pancreatic proteases. Most commonly, protease serine 1 genetic mutations are causative for HP and often result in recurrent early onset episodes of acute pancreatitis typically progressing to chronic pancreatitis, with a high risk of pancreatic cancer. Case Presentation: A 46-year-old female with HP, confirmed by genetic testing, presented with a 7-month history of recurrent pancreatitis. She had previously undergone a distal pancreatectomy and Puestow procedure in 1992 at 21 years of age, after having pancreatitis as a teenager. The patient now had a completion pancreaticoduodenectomy and celiac ethanol nerve block. Conclusion: A completion pancreatectomy in patients with HP can be performed after previous pancreatic surgical intervention to treat disease manifestations and as a prophylaxis against an increased risk of pancreatic adenocarcinoma.