Caroli's disease is a rare congenital disorder characterized by cystic dilatation of the large in-trahepatic bile ducts. The most frequent complications due to biliary stasis are cholelithiasis, cholangitis and sepsis as well as an increased risk of cholangiocarcinoma. Patients may have a history of intermittent abdominal pain, pruritus and/or symptoms of cholangitis. It is rarely diagnosed in childhood. A 12-year-old boy with isolated Caroli's disease is described. This child presented at the age of 2 years, with 4 episodes of recurrent bacterial infections. Interestingly he remained asymptomatic for over 10 years, between the second and third episode. During the 4th episode, when he presented with fever and slight abdominal pain, the diagnosis was made on the basis of radiological findings: U/S, CT, MRI and especially with MRCP, in relation with a more typical picture, resembling cholangitis. Since then he has been followed-up systematically for ten years and remains in good clinical condition without further relapses and with unchanged radiological findings. This atypically benign course of Caroli's disease, with intermittent asymptomatic periods, without any treatment, is very rare.