"The central dot sign is primarily encountered in congenital intrahepatic bile duct dilatation (Caroli syndrome) 1,2. While initially considered specific for this disease, later results have shown that the central dot sign sporadically also occurs in other conditions (e.g. peribiliary cysts, periportal lymphoedema, and jaundice due to biliary obstruction) 3. Nevertheless, the sign aids differentiating Caroli syndrome from other conditions resulting in intrahepatic bile duct dilatation such as primary sclerosing cholangitis or recurrent pyogenic cholangitis 4."