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INTRODUCTION: Choledochal cyst, although a rare disease, is the most frequent malformation of the extrahepatic biliary ducts. The review of the world medical literature gives the overall number of 2500 cases of choledochal cyst. The incidence of this disease is about 1 in 2000000, but it is about four times more frequent in the Yellow race, and 3 times more frequent in females. Congenital choledochal cyst is a disease of paediatric population, as 60% of all patients are younger than 10 years. The basic anomaly of the disease is the embryological arrest of the normal migration of the pancreaticobiliary junction towards the duodenal wall. The result of the disturbance is the extraduodenal location of the pancreaticobiliary junction and the abnormally long common biliopancreatic channel. The flow of bile and pancreatic juice in the duodenal luminary space is insufficient, so the accumulation of bile and pancreatic juice in the common channel and the reflux of this activated mixture towards biliary ducts occur. Choledochal cyst is formed with all its complications: cholangitis, liver abscess, biliary stone formation, biliary cirrhosis, acute and chronic relapsing pancreatitis, and after many years, biliary cancer. Our results in the treatment of this disease are given. We operated on 31 paediatric patients for choledochal cyst over the period 1978-1998. The study is both retrospective and prospective. RESULTS: There were 24 girls and 7 boys, from neonates to teenagers. In 83 percent of patients the complaints started during the first 3 years of life. Unfortunately, only in 22% of patients the complaints were lasting less than 3 months untill the time of operation. The basic complaints were: abdominal pain and jaundice in 95% of patients, vomiting in 74%, high temperature and fever in 56% and palpabile tumour in 48%. About 30% of children had a relapsing cholangitis and 35% of patients an acute or chronic relapsing pancreatitis. Biliary stones were found in 18% of patients. High level of bilirubins was found in 52% of patients, serum transaminase (SGOT and SGPT) in 69%, amylase level was elevated in blood and urine in about half of the patients, and in choledochal cyst in 91%. Ultrasonography was carried out in 78% of patients and has proved to be a very good screening diagnostic tool. Endoscopic retrograde cholangiopancreaticography (ERCP) was performed in most of the patients. DISCUSSION: The only treatment of choledochal cyst is surgical. The conservative treatment is reserved only for complications (pancreatitis, cholangitis). The only adequate surgical treatment is the radical treatment, which consists of the resection of the whole anomalous choledochus and definitive separation of bile ducts from the pancreatic duct. This is achieved by the technique of Roux-en-Y hepaticojejunostomy. The paliative method of internal drainage of choledochal cyst consists of cystoduodeno or cystojejunostomy, but is now abandoned, as the disease continues with all its manifestations and complications. The internal drainage was performed in 6 our patients, but one of them died (a neonate with sepsis at admitance). Three patients were excluded from the study, and 2 girls were later radically reoperated on. In all other 25 patients resection of the whole choledohus and Roux-Y hepaticojejunostomy were carried out. There were neither operative, nor postoperative deaths. Except the temporary bile leaking, spontaneously healed, there were no significant intraoperative complications. All patients were regularly followed-up, they had no complaints, and all their laboratory and ultrasonographic findings were normal. CONCLUSION: Congenital dilatation of biliary ducts is a rare disease, but it must be considered in differential diagnosis of unclear upper abdominal pains, jaundice and pancreatitis in children. In the diagnosis of this disease the ultrasound imaging is of utmost importance as a screening method, and endoscopic retrograde cholangio-pancreaticography as a method of complete visualization. Resection of the diseased common bile duct and Roux-Y hepatico-jejunostomy are the method of choice in the treatment of children with choledochal cyst.