Choledochal cyst is an abnormality of the biliary ducts, characterized by a dilatation of a portion of the biliary ductal system (most commonly a portion or the whole of the extrahepatic bile duct system) that presents usually at ultrasonography as a cystic mass in the right upper quadrant abdomen. Ultrasonographic prenatal diagnosis may be challenging since the detection of any intraabdominal cyst raises the issue of differential diagnosis considering its nature as well as the organ of origin. In this paper, we report our experience with a case of a choledochal cyst strongly suspected during routine midtrimester ultrasound scan, when a round liquid mass was found in the right upper quadrant of the abdomen; the cyst was independent of the other intra-abdominal organs but a connection was observed between the latter cyst and dilated bile ducts. The diagnosis was confirmed at birth and the infant underwent successful surgical correction of the anomaly. We review the current literature as well, discussing the current open challenges of diagnosing and managing these cases.