Abstract:Congenital choledochal cyst (CCC) is the most common congenital biliary malformation clinically with a higher risk of malignancy. Multiple factors, including the age of the patient, cyst type, histological finding and localization, have the impacts on the prognosis. Whether in children or in adults, surgery is the first choice for CCC. The preoperative prediction of canceration, the timing and method of surgery, postoperative complications and the monitoring and management of CCC canceration have the important influences on the prognosis of patients. This article reviews the relevant literature with regards to the prediction, treatment and monitoring of malignant transformation of CCC.