The Hidden Culprit – Caroli Disease Presenting as Recurrent Liver Abscesses in a 5-year-old Boy

Caroli disease is a rare congenital disorder characterized by segmental, nonobstructive dilatation of intrahepatic bile ducts, typically presenting in early adulthood with recurrent cholangitis. Pediatric presentation, particularly with hepatic abscess as the initial manifestation, is exceedingly uncommon. We report a 5-year-old boy who presented with prolonged fever and recurrent abdominal pain, found to have multiple nonresolving liver abscesses despite repeated courses of broad-spectrum antibiotics. Imaging with computed tomography and magnetic resonance cholangiopancreatography revealed segmental intrahepatic biliary dilatation with saccular cystic changes communicating with the biliary tree, confirming the diagnosis of type 1 Caroli disease. The child showed partial clinical and radiological response to prolonged intravenous antibiotics followed by oral prophylaxis, although long-term follow-up was limited. This case highlights an unusual early presentation of Caroli disease and underscores the importance of considering underlying biliary anomalies in children with recurrent or treatment-resistant liver abscesses. Early recognition through appropriate imaging is crucial to guide management and prevent complications such as recurrent infections, biliary stone formation, and malignant transformation.