Objective: To explore the clinical, pathological and imaging characteristics of congenital biliary dilatation (CBD) in children and its management. Methods: The clinical data of 58 pediatric patients with CBD, which was indentified and confirmed by imaging examinations, surgical and pathological findings, were retrospectively analyzed. Results: All the 58 patients exhibited clinical symptoms that mainly were abdominal pain, jaundice and abdominal mass, and some of them were combined with fever and vomiting. All the results of CT (performed in 45 cases), ultrasonography (performed in 40 cases) and magnetic resonance cholangiopancreatography (MRCP) (performed in 17 cases) revealed bile duct dilatation, and 11 cases were diagnosed with pancreaticobiliary maljunction (PBM) by MRCP. Intraoperative cholangiography was successfully performed