<prompt> All are true regarding carolis disease exceptCholangitisAssociated with congenital hepatic fibrosisCommonly presents with ascitesMalignant change can occur  <response>: CONGENITAL DILATATION OF THE INTRAHEPATIC DUCTS CAROLIS DISEASE This rare congenital condition is characterised by multiple irregular saccular dilatations of the intrahepatic ducts, sepa- rated by segments of normal or stenotic ducts, with a normal extrahepatic biliary system. In Carolis syndrome, the biliary dilatation is associated with congenital hepatic fibrosis. The presentation is varied, with many patients presenting with abdominal pain, cholangitis or end-stage liver disease. The majority of patients present before the age of 30. Sex distri- bution is equal. Management is multidisciplinary: cholangitis or jaundice are treated with appropriate antibiotic therapy and endoscopic or interventional stenting. Malignancy is a complication of long-standing disease. Hepatic resection is indicated for patients with limited disease. Patients with dif- fuse disease and concomitant hepatic fibrosis are candidates for liver transplantation. Recurrence is common, paicularly after resection, and long-term surveillance is required. Ref: Bailey and love 27th edition Pg no :1200 