Caroli’s disease and Caroli’s syndrome are rare congenital cystic disorders of intrahepatic biliary radicals. It is still unclear whether they represent distinct entities or, are different stages of the same disease distinguished by hepatic fibrosis. Here we are reporting two cases-a three-year-old boy presenting with portal hypertension, cholestatic jaundice and rickets, and a ten-year-old girl with recurrent episodes of cholangitis. They were diagnosed as Caroli’s syndrome and Caroli’s disease respectively. A renal calculus with medullary sponge kidney was detected on abdominal imaging in second case. We concluded that they are probably the different stages of the same disorder and ongoing search should be continued to quantify accurately the disease spectrum, and its associations.
