Onset of symptoms occurred from 7 days of age to 9 5 years; in 10 patients onset occurred under 12 months of age, in 3 from 13 to 24 months, and in the remaining 3 from 2 to 9*5 years. The first symptoms were jaundice, vomiting, acholic stools, and poor sucking. Obstructive jaundice and acholic stools were found in more than 80 % of the patients, and abdominal mass in 60 %. Vomiting occurred in 50% of the patients, one-third of whom showed failure to thrive, and 70% of patients over the age of 1 -5 years had abdominal pain. About half of the patients had a mild to moderate degree of fever.In all patients primary excision of the cyst was followed by hepaticojejunostomy in Roux-en-Y fashion. The postoperative course was uneventful in 14 patients; 2 patients died, one from ascending cholangitis and one from hepatic failure.