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. This evidence concerns the gene MSH2 and paramyotonia congenita of Von Eulenburg.