Caroli syndrome is a rare condition and is composed of congenital cystic dilatation of the biliary system and congenital hepatic fibrosis. Although many associated conditions are defined and hypothesized to occur concomitantly, due to the rarity of this syndrome, none has proven to be an essential component of this syndrome. In order to investigate a patient presenting with a cholestatic clinical picture, ultrasound, endoscopic retrograde cholangiopancreatography, abdominal computed tomography, liver biopsy, splenoportal venous angiography, and all available liver tests were performed. Upon typical findings, a diagnosis of Caroli syndrome was made and an orthotopic liver transplantation was performed. Investigation of the patient demonstrated multiple intracystic stones mimicking hemangiomatosis in the ultrasound; severe irregularity and narrowing in the main bile duct mimicking sclerosing cholangitis in the endoscopic retrograde cholangiopancreatography; partial portal vein thrombosis with irregularity in the portography; and a unilobar cirrhosis of the left liver lobe while the right lobe demonstrated only congenital hepatic fibrosis in the explanted liver. Caroli syndrome may be associated with main bile duct and portal vein abnormalities. Although the syndrome can be monolobar in nature, a cirrhotic left lobe sparing the right lobe, partially affected by the cirrhotic process, has never been defined. Here, we report a case of Caroli syndrome who had liver transplantation, with very rare and interesting findings of the explanted liver, such as tapering cirrhosis from the left lobe to the right lobe and countless stones in biliary cysts mimicking hemangiomas.