Choledochal cyst (CC) is a common congenital biliary tract disorder in pediatric patients, with abdominal pain, abdominal mass, and jaundice as the main clinical manifestations. The disease requires early surgery once diagnosed to alleviate biliary tract infection and biliary cirrhosis caused by obstructive jaundice. The clinical manifestation of choledochal cyst depends on the degree of obstruction of the distal common bile duct; the more severe the obstruction, the more severe the manifested symptoms and the earlier the appearance of symptoms. There are different clinical manifestations at different ages of onset, with neonates and young children usually presenting with abdominal masses, obstructive jaundice and white stools. In severely obstructed cases, they may present with manifestations similar to biliary atresia, such as severe jaundice and white stools, and some present with a large epigastric mass without jaundice. Older children usually present with the classic triad of abdominal pain, abdominal mass and jaundice, with abdominal pain predominating, while fever and vomiting also occur. The abdominal pain resembles the symptoms of recurrent pancreatitis and is accompanied by high levels of pancreatic amylase in the serum in some cases. In adult-onset cases, the degree of dilatation of the common bile duct is usually mild, mainly manifesting as abdominal pain and symptoms of chronic cholecystitis. Some patients develop gallbladder cancer because the concentrated pancreatic fluid in the gallbladder irritates the gallbladder mucosa, resulting in eventual malignancy. For choledochal cysts found prenatally, once they are found, they should be treated promptly after birth. Because prenatal choledochal cysts are likely to lead to progressive liver damage, if left untreated, there is a risk of cirrhosis of the liver. All children with prenatally detected choledochal cysts should receive surgical treatment regardless of whether they have symptoms or not.