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The clinical features and long-term outcome of 21 children with choledochal cyst treated over a 31-year-period is reviewed. All 7 infants ( < 1-year-old) presented with jaundice. Only 3 older children (14%) presented with the classical triad of pain, jaundice and an abdominal mass. Other forms of presentation included pancreatitis (n = 8), cholangitis (n = 3), biliary peritonitis (n = 2) and biliary cirrhosis (n = 2). An incidental diagnosis of a choledochal cyst was made in 3 patients one each with bilateral ureteroceles, renal hypoplasia and meningitis with hepatitis A infection. An elevated serum amylase (SA: mean = 1005 U/L) and intraoperative bile amylase (BA: mean = 16,902 U/L) was observed in all 8 children with pancreatitis. Complete excision of the choledochal cyst with Roux-en-Y hepaticojejunostomy was the primary operative procedure in 18 patients. The remaining patients underwent cystoduodenostomy (n = 2) and cystojejunostomy (n = 1). Recurrent cholangitis and stricture formation complicated cyst enterostomies. In comparison cyst excision with Roux-en-Y hepaticojejunostomy gave excellent long-term results with minimal complication.