Germline pathogenic variations were detected in 15 of 355 patients with acute pancreatitis (4.2%), including a recurrent variation of SPINK1 c.194 + 2T>C in 11 patients (3.1%), CFTR variations (p.Arg74Trp and p.Gly970Asp) in 2 patients (0.6%), and cancer susceptibility gene variations (MLH1 p.Ser577 = and ATM p.Phe2558fs) in 2 patients (0.6%). Here, CFTR is linked to acute pancreatitis.