Several tumor syndromes are known to have an increased incidence of pancreatic carcinoma. Unfortunately, the incidence of pancreatic carcinoma almost equals its mortality. Because of intense research activities in the past decade, several genetic alterations have been identified that are responsible for the so-called sporadic pancreatic carcinoma development. National and international research activities only recently have begun focusing on the molecular pathogenesis of hereditary risk factors for pancreatic carcinoma. It is hoped that these genetic studies will aid the development of clinical management strategies for pancreatic carcinoma prevention.