Our study is the first to report a significant association between the L78R CHDH SNP and the risk of pancreatic cancer, where we found that the LL genotype of the polymorphism L78R CHDH was significantly protective (OR = 0.29; CI 0.12–0.76) against pancreatic cancer when compared with the referent RR allele. The gene discussed is CHDH; the disease is familial pancreatic carcinoma.