A case of intraductal papillary-mucinous neoplasm (IPMN) of the pancreas associated with polycystic liver and kidney disease is reported. A 63-year-old man was admitted to hospital with heartburn and upper abdominal pain. CT indicated multiple cysts in the liver and bilateral kidneys, and also showed multiple cystic lesions in the pancreatic head. Pancreatoduodenectomy was performed, and a pathological diagnosis of intraductal papillary-mucinous adenoma of branch duct type of the pancreas was made. He died of renal failure and hepatic failure due to recurrent cholangitis 12 years after the operation. An autopsy confirmed multiple cysts in the liver and kidneys. Multiple hepatic cysts were lined by biliary-type epithelium with no connection to the bile ducts. Peribiliary cysts were observed, and histology of the liver indicated von Meyenburg complexes. The kidneys showed that the cysts originated from all segments of the renal tubule. The autopsy findings suggested that the patient had suffered from autosomal-dominant polycystic kidney disease (ADPKD), but he did not have a family history of ADPKD. This case demonstrates that IPMN of the pancreas can occur as an extrarenal complication in patients with polycystic liver and kidney disease.