We conducted a case-control study to investigate the association between the survivin rs9904341 G<C polymorphism and risk of pancreatic cancer. A total of 261 pancreatic cancer patients who were pathologically confirmed to have pancreatic cancer were included in the study. Polymorphisms of rs9904341 G<C were assessed by polymerase chain reaction-restriction fragment length polymorphism. Pancreatic cancer patients were more likely to be smokers, have a family history of cancer, and have diabetes. Using multivariate logistic regression analysis, the CC genotype was found to be associated with a significantly increased risk of pancreatic cancer risk compared with the GG genotype (odds ratio = 1.85, 95% confidence interval = 1.12-3.01). Moreover, those carrying the C allele had a higher risk of pancreatic cancer compared to those with the G allele (odds ratio = 1.38, 95% confidence interval = 1.08-1.77). In conclusion, we found that the survivin rs9904341 polymorphism was associated with an increased risk of acute pancreatitis.