Carols disease with congenital hepatic fibrosis was diagnosed in a 34-year-old woman. She com- plained of sudden hematemesis and easy fatigability. She showed a esophageal varix, hepatomegaly and splenomegaly, but showed normal liver function test. We could not find peripheral stigmata of liver disease such as palmar erythema and spider angioma. Abdominal sonogram, ERCP, abdominal CT and celiac angiogram showed multiple intrahepatic ducts dilatations, characteristics of Carolis disease. Histologic finding of liver biopsy showed fibrous tracts containing dilated bile ductules connecting adjacent portal spaces that were widened by mature fibrosis tissue and liver parenchymal cells appeared normal. We should suspect the Carolis disease with congenital hepatic fibrosis those who showed hematemesis or cholangitis with normal liver function test and no peripheral stigmata of liver disease. As Caroli's disease combined congenital hepatic fibrosis frequently. Carolis disease was preoperatively diagnosed by radiologic study such as ultrasonography, ERCP and abdominal CT but congenital hepatic fibrosis was diagnosed only by liver biopsy. We presented a Carolis disease combined with congenital hepatic fibrosis and renal tubular ectasia with literature.