Caroli cyst presenting as recurrent biliary colic with cholesterol cholelithiasis: A case report and review of the literature

Caroli disease is a rare congenital disorder characterized by segmental, nonobstructive dilatation of the intrahepatic bile ducts. Its association with cholesterol cholelithiasis is infrequently reported and presents a diagnostic challenge, particularly when clinical findings and conventional imaging are inconclusive. We report the case of a 45-year-old woman, nonsmoker, with no prior medical or surgical history, who presented with 2 episodes of severe abdominal pain. Initial workup, including abdominal ultrasonography and liver function tests (LFTs), was unremarkable on first presentation, yet LFTs were elevated on both admissions. Repeat ultrasonography remained negative. Magnetic resonance cholangiopancreatography (MRCP) excluded choledocholithiasis but identified a dilated left intrahepatic biliary duct. Endoscopic ultrasound (EUS) subsequently confirmed cholesterol gallstones in association with a Caroli cyst. The patient underwent laparoscopic cholecystectomy with an uneventful postoperative course and was discharged 2 days later in good clinical condition. This case underscores the diagnostic complexity of Caroli cysts coexisting with cholesterol cholelithiasis and highlights the critical role of advanced hepatobiliary imaging, particularly MRCP and EUS, when standard investigations fail to explain recurrent biliary symptoms. Clinicians should maintain a high index of suspicion for congenital biliary anomalies in patients with unexplained recurrent abdominal pain and elevated LFTs.