Cholangiocarcinoma has been associated with various fibrocystic diseases of liver and biliary tract, but cholangiocarcinoma in polycystic liver disease (PLD) was extremely rare. It was reported that the prognosis of cholangiocarcinoma associated PLD was very poor and distant metastases were common. We report a rare case of intrahepatic cholangiocarcinoma associated with PLD in 58-year-old female who presented vague abdominal pain. She had also polycystic kidneys with normal renal function. MRI showed well demonstrated tumor with central scar and a adjacent large cyst in the left lobe of the liver. She underwent extended left lobectomy. On microscopic examination, the tumor was moderately differentiated cholangiocarcinoma having abundant fibrous stroma and necrosis and the adjacent cyst showed focal in situ carcinomatous changes in the lining epithelium. (Korean J Hepatol 1999;5:156－161)
