Though hereditary pancreatitis is a very rare form of pancreatitis, the discovery of the gene for hereditary pancreatitis provides important implications for pancreatitis in general. A premature activation of trypsinogen is likely to occur not infrequently in our daily life as the onset of the disease is well advanced before a drinking habit starts. It probably goes unnoticed in normal individuals because normal inhibitory mechanisms described above prevent the development of pancreatitis. Any disorders or agents that cause abnormalities in this natural protective mechanism can cause pancreatitis. Genotype-phenotype analyses of CFTR mutations in chronic pancreatitis are necessary to establish the relationship between CFTR and this disease. It remains to be shown how a reduction of functional CFTR causes chronic pancreatitis.