BACKGROUND Choledochal cysts are dilations of the biliary tree. Although commonly reported in Asian populations, the incidence outside of Asia is as low as 1:150 000. The largest series of patients with choledochal cyst disease outside of Asia is this one, studying 70 patients treated in Vancouver between 1971 and 2003. METHODS This was a retrospective chart review. RESULTS In all, 19 paediatric and 51 adult patients were evaluated; 21% of paediatric and 25% of adult patients were Asian. All paediatric patients had type I or IV cysts, whereas adult patients represented the different subtypes. Abdominal pain was the presenting symptom in 79% of children and 88% of adults, vomiting was present in 42% of children and 63% of adults and jaundice was seen in 31.5% of children and 39% of adults. Ultrasound was used in 94.7% of children, and ERCP in 80% of adults. In all, 84% of paediatric patients, 100% of adult patients with type I cysts and 85.7% of adult patients with type IV cysts received complete cyst excision and Roux-en-Y hepaticojejunostomy. Complications in both groups were low. CONCLUSIONS Although Vancouver does have a large Asian population, this does not explain how common choledochal cysts are in this city. Although some authors argue that paediatric and adult disease are caused by different aetiologies, presentation patterns in our study between the two groups were very similar. We recommend complete cyst excision and Roux-en-Y hepaticojejunostomy as the surgery of choice, and advocate early surgery after diagnosis to promote ease of surgery and prevention of future complications.