A 12-month-old boy presented with fever, Klebsiellabacteremia, emesis, and abdominal pain. US demonstratedmultiple intrahepatic cysts containing fibrovascular bundles,known as the central dot sign (Fig. 1). Subsequent percuta-neous transhepatic cholangiography showed segmentalsaccular biliary ductal dilation (Fig. 2).Caroli disease is a rare congenital cystic dilatation of theintrahepatic bile ducts. This predisposes to biliary stasis andconsequent lithiasis, cholangitis, abscesses, and septicemia.Additionally, children face increased risk of hepatic fibrosis(termedCarolisyndrome),renaldisease,cholangiocarcinoma,and amyloidosis [2]. The central dot sign on US and CT isindicative of Caroli disease, but can also be seen inperibiliary cysts, obstructive jaundice, and periportal lym-phedema [1]. Cholangiography is the most sensitive radio-graphic study, because communication between bile ductsand cystic loculations is important to differentiate frommultiple liver cysts [2]; the role of magnetic resonancecholangiopancreatography for Caroli disease in children hasnot been systematically studied. Treatment can be achievedby partial hepatectomy in localized disease; however, diffuseliver involvement necessitates eventual transplant.References