Intraductal papillary mucinous neoplasms and PNETs may be associated in a number of scenarios. The association may be due to the concurrent existence of independent lesions, may be a mixed endocrine-exocrine tumor, or may be due to intraductal growth of the endocrine lesion. But the literature is confusing. It is not known whether the association is accidental or whether there is an etiological reason. Further studies are needed to investigate this scenario.