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Choledochal cyst is an uncommon congenital cystic dilatation of the bile duct. The underlying etiology is believed to be an anomalous junction of the pancreatic duct and common bile duct (CBD) that allows free reflux of pancreatic enzymes into the CBD, weakening its wall. Portal hypertension is a rare complication of choledochal cyst. We report a case of choledochal cyst with portal hypertension confirmed by surgery