The proposed diagnosis was mixed IPMN with associated invasive medullary carcinoma that presented loss of MSH2 and MSH6 expression.<h4>Conclusions</h4>The present case reports for the first time, at the best of our knowledge, the coexistence of IPMN lesions and PMC, both having the same molecular alterations. Here, MSH6 is linked to pancreatic intraductal papillary-mucinous neoplasm.