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Liver cysts in children are uncommon abnormalities which can now be easily diagnosed by means of modern technical diagnostic imaging and treated in due course. Choledochal cysts are rare congenital cystic dilatations of the biliary tract, which can involve the intra- or extrahepatic bile ducts. The estimated incidence in North America is 1: 150,000 live births. This infrequent anomaly occurs more commonly in Asian female infants. This article presents a rare congenital case of choledochal cyst. The patient is a 16 month female infant in Zahedan, who was taken to the pediatric ward of Ali ebne Abutaleb hospital by her parents. Her chief disorder was a progressive abdominal mass reported by her parents. In physical examination a palpable right upper quadrant abdominal mass was found in the absence of jaundice and achromatic stool. Abdominal radiography showed a dilated extrahepatic duct and a sub-hepatic cystic mass with 110×85×97 mm dimensions and 400 cc volume, which was confirmed by abdominal MRI and MRCP techniques. The patient underwent an exploratory laparatomy, which revealed a type III choledochal cyst (choledochocele). The cyst was resected completely and the biliary tract was bypassed. The patient was discharged while she was in good general condition and her following up showed no post -operative complications such as malignancy, stenosis, cholangitis and cholelithiasis.

     

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