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Caroli disease was first described in 1958. It is characterized by dilatation of intrahepatic bile ducts. There are two variants of the disease: Caroli disease and Caroli syndrome. In the latter, congenital hepatic fibrosis is present in addition to dilatation of the ducts. The disease is closely associated with autosomal recessive polycystic kidney disease. The main pathological feature is improper remodeling of the ductal plate. Symptoms are non-specific and in most cases occur in childhood or in youth. Patients have pain in the upper right quadrant of the abdomen, fever, itching and fatigue. In clinical examination, enlarged liver and enlargement of the spleen are found. Formation of biliary stones, cholangitis, sepsis, and biliary cirrhosis are common complications. Diagnosis of the disease is established with imaging diagnostics–ultrasound, endoscopic retrograde cholangiopancreatography, magnetic resonance cholangiography, CT, and MRI. Treatment is symptomatic with the aim of treating and preventing the complications of the disease. The final solution is liver transplantation. Survival rates after transplantation are high. Patients with Caroli disease or Caroli syndrome are at an increased risk of cholangiocarcinoma; screening of asymptomatic patients is therefore advisible.