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Background: The aim of this study was to report a Western experience in the diagnosis and management of choledochal cyst disease.Results: Sixty-seven patients were identified including 15 children and 52 adults; 76.1 % were females. The median age at diagnosis was 3 [inter-quartile range (IQR) = 6.0-0.7] years for children, and 46 [IQR = 55.6-34.3] years for adults. Forty-eight patients (72 %) were symptomatic. Types of choledochal cyst included: I (n = 49, 73.1 %), II (n = 1, 1.5 %), IV (n = 9, 13.4 %), and V (n = 8, 12 %). The median diameter of the type I choledochal cyst was 35 [IQR = 47-25] mm. All 48 patients underwent excision of cyst with Roux-en-Y hepaticojejunostomy, and eight underwent resection with hepaticoduodenostomy. Six patients underwent liver resection, and five patients underwent orthotopic liver transplantation. Malignancy was concomitant in five adult patients, being identified on preoperative imaging in three cases; and atypia was seen in three additional patients. Early morbidity included Clavien-Dindo classification grades III (n = 7) and II (n = 5), while long-term complications consisted of Clavien-Dindo grades V (n = 5), IV (n = 2), III (n = 18), and II (n = 1).Conclusions: Presentation and management of choledochal cyst is varied. Malignant transformation is often detected incidentally, and so should be the driving source for resection when a choledochal cyst is diagnosed. [ABSTRACT FROM AUTHOR]