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Context Acute pancreatitis has occasionally been reported in association with Campylobacter jejuni infection in humans. However, the mechanism linking Campylobacter jejuni infection and pancreatitis isunclear. Acute pancreatitis in association with an infectious illness may be related to underlying genetic mutations. For instance, studies show that mutations in the cystic fibrosis transmembrane conductance regulator gene increase the susceptibility for acute and chronic pancreatitis. Case report We describe a patient with Campylobacter jejuni infection who developed acute pancreatitis in the setting of an underlying cystic fibrosis transmembrane conductance regulator gene mutation. Discussion In this patient with an underlying mutation in the CFTR gene, we propose that the interaction between the mutant gene and an environmental factor, Campylobacter jejuni infection, resulted in pancreatitis.