Hereditary pancreatitis is presently receiving much attention and deservedly so, not only because of the identification of three genes in separate families, but also because of the unraveling of at least one pathogenetic mechanism. At this point, it is useful to review some historical high points in understanding of this condition. Although familial pancreatitis is a generic term encompassing many family members affected with pancreatitis, with or without a genetic defect (examples include alcoholic pancreatitis or hyperlipidemia), hereditary pancreatitis relates to a specific genetic defect identified in all the members of a family affected with pancreatitis. Three genetic abnormalities are reviewed in greater detail.