A 36-year-old woman with a history of a Todani type IV choledochal cyst (CC), for which she had previously undergone a cystoduodenostomy when she was 13 years old, was admitted and treated for cholangitis. Laboratory investigations revealed a total bilirubin of 31 μmol/L, serum alkaline phosphatase of 70 IU/L, white cell count of 14.08 cells/L, and carbohydrate antigen 19-9 U/mL of 68. Abdomen ultrasonography showed cystic dilatation of the biliary tree. Endoscopic retrograde cholangiopancreatography was unsuccessful. Magnetic resonance cholangiopancreatography (MRCP) showed multiple biliary strictures with ductal dilatation and ductal stones. It also revealed cystic dilatation in the common bile duct, with a maximum diameter of 2.1 cm and filling defect distally (Figure A). Percutaneous transhepatic cholangiography (PTC) was performed, which showed filling defects within the cystoduodenostomy suspicious of malignancy (Figure B). Fusion positron emission tomography/computed tomography (PET/CT) subsequently performed by using 8.2 mCi of [18F-fluorodeoxyglucose] administered intravenously confirmed a metabolically active focus within the cystoduodenostomy corresponding to the location of the filling defect seen at PTC, with a standardized uptake value of 4.6 suggestive of malignancy (Figure C). At surgery, a tumor was found within the 3 × 2.5 × 2 cm CC. En-bloc resection of the CC and cystoduodenostomy including pancreaticoduodenectomy with reconstruction via a double-duct hepaticojejunostomy was performed, and the patient recovered well. Final pathologic examination confirmed a moderately differentiated adenocarcinoma arising within the previous cystoduodenostomy and invading into the muscular wall of the duodenum (Figure D). The diagnosis of a CC often involves multiple imaging modalities.1Corvera C.U. Blumgart L.H. Akhurst T. et al.18F-fluorodeoxyglucose positron emission tomography influences management decisions in patients with biliary cancer.J Am Coll Surg. 2008; 206 (Epub 2007 Oct 1): 57-65Abstract Full Text Full Text PDF PubMed Scopus (197) Google Scholar, 2Park D.H. Kim M.H. Lee S.K. et al.Can MRCP replace the diagnostic role of ERCP for patients with choledochal cysts?.Gastrointest Endosc. 2005; 62: 360-366Abstract Full Text Full Text PDF PubMed Scopus (93) Google Scholar Presently, MRCP might be considered the diagnostic modality of choice because it is noninvasive and demonstrates excellent visualization of the biliary tree.2Park D.H. Kim M.H. Lee S.K. et al.Can MRCP replace the diagnostic role of ERCP for patients with choledochal cysts?.Gastrointest Endosc. 2005; 62: 360-366Abstract Full Text Full Text PDF PubMed Scopus (93) Google Scholar Although previously cystoenterostomy without resection was a popular operation for CC, this is no longer recommended because of the risk of carcinoma developing within the cyst wall, as illustrated by this case. Presently, the management of choice of a CC is complete excision of the entire cyst including all at-risk mucosa to prevent malignant transformation within the remnant wall. Preoperative imaging for cholangiocarcinoma is imperative in assessing the resectability of the tumor, because most patients present late with locally or systemically advanced disease. Several studies have demonstrated the utility of PET/CT in the diagnosis and staging of cholangiocarcinomas that are not diagnosed on standard imaging.3Anderson C.D. Rice M.H. Pinson C.W. et al.Fluorodeoxyglucose PET imaging in the evaluation of gallbladder carcinoma and cholangiocarcinoma.J Gastrointest Surg. 2004; 8: 90-97Crossref PubMed Scopus (288) Google Scholar However, to date, none has reported the use of PET/CT in the detection of malignancy within CCs. The present case is unique because it demonstrates the use of PET/CT in the diagnosis of cholangiocarcinoma arising within a CC. Accurate preoperative diagnosis was important in our patient so that surgical resection with good oncologic clearance was performed. We would like to thank Drs Richard Lo, MBBS, FRCR and Foong-Koon Cheah, MBBS, FRCR from the Department of Radiology, Singapore General Hospital for their assistance in interpreting the radiological images.