Caroli's syndrome.

We report the case of a two-year-old girl presenting with recurrent episodes of fever and hepatomegaly. High resolution ultrasound (US) and computed tomography (CT) of the liver show dilated intrahepatic bile ducts with bridge formation and intraluminal protrusions. The extrahepatic bile duct is normal. The diagnosis of irregular dilated bile ducts with signs of cholangitis and cholangiolitis is proved by liver biopsy. The histologically associated congenital liver fibrosis in our patient enter into the Caroli's syndrome. We illustrate the high resolution US and the CT findings in this case.