Acute and chronic pancreatitis and pancreatic cancer represent the major disease of the pancreas in humans. The etiology, mechanisms of progression, early detection, early diagnosis, treatment and preventative strategies remain poorly defined and understood. Furthermore, there appears to be a connection between acute pancreatitis, chronic pancreatitis and pancreatic cancer. Environmental factors play an important, but limited role. Alcohol abuse and tobacco smoking are powerful cofactors for pancreatic disease, but are insufficient to cause or drive disease progression alone. Few other toxic factors have been identified, possibly because the pancreas is protected form direct contact from the environment in contrast to the skin, lungs and digestive tract, and is not involved in detoxification or elimination of waste as in the case of the liver, kidneys, bladder or colon. A major breakthrough in understanding pancreatic diseases came from the discovery that mutations in the trypsinogen gene. Within the last few years the importance of several genetic factors has emerged, especially mutations in CFTR, the SPINK1 gene and others. These genetic factors appear play a role in the initiation and severity of acute pancreatitis, idiopathic chronic pancreatitis in children and adults, tropical pancreatitis, alcohol-related chronic pancreatitis, and the development of pancreatic cancer. Additional breakthroughs in animal models, developmental genes, and the cause of Shwachman- Diamond syndrome have recently emerged. This new and emerging knowledge has profound clinical implications, and issues of genetic testing and counseling must also be discussed. Based on these time sensitive and important issues we are proposing the Fourth International Symposium on Inherited Diseases of the Pancreas to be held in Chicago, Illinois, immediately following the American Pancreatic Association Annual Meeting (November 7-9, 2003). The program is organized and sponsored by the Midwest Multicenter Pancreatic Study Group (MMPSG), which represents academic pancreatologists from major universities throughout the United States and several locations in Europe. The format, venue, and content are designed to maximize participation, education, translation and implementation of the key elements of the many new findings in the genetics of the pancreas.