Hereditary pancreatitis is a rare type of acute or chronic pancreatitis with clinical symptoms similar to pancreatitis caused by other causes. In 1996, Whitcomb firstly reported that mutation of PRSS1 is the cause of hereditary pancreatitis. Subsequently, SPINK1, CFTR and CTRC were reported to be associated with hereditary pancreatitis, but whether they are the causative genes of hereditary pancreatitis remains controversial. PRSS1 encodes cationic trypsinogen, and mutations in this gene may result in increased trypsin activation or reduced inactivation, causing clinical pancreatitis. More than 20 pathogenic mutations of PRSS1 have been reported, and the most common mutation sites are R122H, N29I and A16V. The onset of hereditary pancreatitis occurs at a younger age, and usually before the age of 20, with an average onset age of 10 years. The average age for developing chronic pancreatitis is 20 years, and the risk for progressing to pancreatic cancer is dramatically increased after 
50 years of age. Management of patients with hereditary pancreatitis includes avoidance of environmental triggers, therapy for endocrine and exocrine insufficiency, pain management, endoscopic or surgical treatment and surveillance for pancreatic cancer. Here, the authors mainly address the research progress of the pathogenesis and treatment as well as disease management of PRSS1-related hereditary pancreatitis.