Although IPMN shares some common genetic mutations with pancreatic infiltrating ductal adenocarcinoma (e.g., KRAS proto-oncogene, GTPase (KRAS), and tumor protein P53 (TP53) and cyclin-dependent kinase inhibitor 2A (CDKN2A)), it additionally comprises unique mutations in the guanine nucleotide-binding protein-stimulating α subunit (GNAS; 41–79%) [31,32,33] and ubiquitin E3 ligase ring finger 43 (RNF43; 38%) [34,35], which are putatively related to the pathogenesis of IPMN. Here, RNF43 is linked to pancreatic intraductal papillary-mucinous neoplasm.