Intraductal papillary mucinous neoplasms of the pancreas (IPMN) are relatively rare, mucin-producing pancreatic neoplasms that are visible grossly within the pancreatic ductal system and usually form papillary structures. Four main directions of differentiation of IPMN are described: intestinal, pancreaticobiliary, oncocytic and gastric-foveolar (GF-IPMN). We report a histopathological and immunohistochemical study of GF-IPMN which involved the main pancreatic duct and progressed to invasive carcinoma. The presented case of invasive carcinoma derived from IPMN indicates that the dichotomous model of IPMN progression (intestinal pathway or pancreaticobiliary pathway) is inadequate in some IPMN. Additionally, we describe the issues of GF-IPMN epidemiology, molecular characteristics and differential diagnostics.