Chronic pancreatitis (CP) is a persistent inflammation of the pancreas, usually with chronic or recurring attacks of abdominal pain. CP results in permanent changes in the structure of the pancreas and leads to endocrine and exocrine insufficiency. The prevalence of CP is from 3 to 30 cases per 100,000 individuals. CP can have a number of causes, including toxic (most often alcohol), idiopathic (unexplained), genetic, and other forms. Hereditary pancreatitis (HP) is inherited in families in an autosomal dominant inheritance fashion and accounts for approximately 1% of all CP. A diagnosis of HP is made if three or more cases of CP occurred in the same family across two or more generations, and there is equal gender distribution. Diagnosis of CP is made using clinical presentation, assessment of pancreatic function using laboratory tests, and the results of imaging studies. CP can be difficult to differentiate from recurrent acute pancreatitis during its early stage. Treatment of CP is mainly focused on management of symptoms, especially of pain, and on endocrine and exocrine insufficiency. Patients with HP have a 40% lifetime risk of pancreatic cancer. Dietary changes to reduce meal size and lower fat consumption are important, as are lifestyle modifications to reduce or eliminate consumption of alcohol and tobacco. HP is mainly associated with sequence variants in the protease, serine, 1 (trypsin 1) gene (PRSS1), which encodes the cationic trypsinogen, a key pancreatic enzyme. Although rarely found in patients with HP, sequence variants in three other genes may confer an increased risk for developing pancreatitis. These three genes are the serine peptidase inhibitor, Kazal type 1 gene (SPINK1), the chymotrypsin C (caldecrin) gene (CTRC), and the cystic fibrosis transmembrane conductance regulator (ATP-binding cassette sub-family C, member 7) gene (CFTR), which is more commonly associated with cystic fibrosis.