Acinar cell carcinoma (ACC) is a rare pancreatic neoplasm with dismal prognosis. Insights into the molecular basis of ACC can pave the way for the application of more effective, personalized therapies and detection of patients with hereditary predisposition. Molecular analysis revealed a germline (and ) mutation in a patient with a rare pancreatic ACC with extensive intraductal growth. Somatic loss of the wild-type allele in the tumor indicated the causal relationship of ACC with the germline defect. A thorough literature review identified another nine ACCs associated with germline mutation and two ACCs associated with germline mutation, resulting in a prevalence of germline mutations in almost 7% of ACCs. Moreover, somatic alterations are reported in 16% of sporadic ACCs. Overall, about one fifth (22%) of all pancreatic ACCs exhibit BRCA1/2 deficiency. This study underscores the important role of mutations in pancreatic ACC. All ACC patients should undergo genetic testing for mutations to identify carriers of pathogenic variants. This will allow to select patients that can benefit from targeted therapies directed against -deficient tumors and is also crucial as a referral to genetic screening for the relatives of affected individuals carrying germline alterations. ACC: acinar cell carcinoma; HBOC: Hereditary Breast and Ovarian Cancer; LOH: loss of heterozygosity; PARP: poly (ADP-ribose) polymerase; PDAC: pancreatic ductal adenocarcinoma; PP: pancreatic panniculitis; SD: standard deviation; WES: whole-exome sequencing.