Objective.The aim of this paper is to describe the mechanism, clinical manifestation, diagnosis and surgical management of congenital choledochal cyst. Methods. From 1984 to 1997, 56 cases of congenital choledochal cyst were reviewed. Among them, 8 patients were male, 48 patients were female, the age ranges from 12 to 50 years old with an average of 26.3.Results.The main clinical manifestation includes jaundice, abdominal pain and abdominal mass. All of them were confirmed by ultrasonography, while 39 cases were performed ERCP and PTC with the same diagnosis. Fifty-one patients were performed cystectomy and hepatojejunostomy, two cases were performed cystjejunostomy because of diffused angioma and severe hemorrhage respectively, external drainage was performed in one case with emergent cholangitis, the other two cases were reported malignancy through biopsy and operation was abandoned.Conclusions. Ultrasound diagnostics is essential to accurately diagnose the cyst, preoperative ERCP is helpful for differentiating pancreatic duct from bile duct, while MRCP is a reliable method; cystectomy and cholangiojejunostomy is recommended, laparoscopic procedure is becoming more and more accepted.