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Pancreaticoduodenectomy for choledochal cyst. BACKGROUND/AIMS: Choledochal cyst (CDC) is a rare congenital cystic dilatation of the biliary system that can affect children and adults. Standard treatment for CDC includes surgical excision of the cystic bile duct with restoration of bilioenteric continuity by Roux-en-Y hepaticojejunostomy. In rare situations, however, surgical excision of a CDC may require pancreaticoduodenectomy (PD). The goal of this study was to identify instances where a PD may be deemed necessary. METHODOLOGY: We retrospectively reviewed the medical records of all patients treated for CDC at our institution from January 1988 through December 2011. From this cohort, patients who underwent PD were reviewed in detail. Data on preoperative parameters, operative details, cyst type, pathologic diagnosis, and postoperative course were collected. RESULTS: During the 24-year period, 59 patients with CDC were identified and four (7%) underwent formal PD. Final pathology revealed that none of the resected specimens harbored malignancy and all patients are well at follow-up. CONCLUSION: While the standard surgical care for CDC remains complete cyst excision with Roux-en-Y hepaticojejunostomy, there may be particular instances where PD appears more appropriate. While the need for PD in cases of CDC is rare, it is important to consider this possibility when contemplating surgery for CDC.