ABSTRACT
Caroli disease is a rare congenital disorder of segmental cystic dilatations of the intrahepatic biliary ducts that maintain communication with the rest of the biliary tree. Its typical clinical presentation is recurrent episodes of cholangitis. The diagnosis is usually made using abdominal imaging modalities. We present a patient with Caroli disease showing atypical presentation of acute cholangitis with ambiguous labs and initial negative imaging findings, which was later diagnosed by [18F]-fluorodeoxyglucose positron emission tomography/computed tomography and confirmed by magnetic resonance imaging and tissue pathology. The use of such imaging modalities in moments of doubt or clinical suspicion provide the patient with accurate diagnosis, proper management, and better clinical outcome, thus obviating the need for further invasive investigations.
