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Introduction: Portal vein (PV) disorders are various, but rare. Here, we report a preduodenal superior mesenteric vein (PDSMV) in a patient who underwent a pancreaticoduodenectomy., Presentation of Case: A 67-year old woman with familial adenomatosis polyposis was suspicious for cancer of the papilla of vater and scheduled for surgery. Pre-operative diagnostic revealed a PDSMV continuing into the left PV. The splenic vein (SV) continued directly into the right PV without forming ananatomic PV confluence. Eight centimetre of the PDSMV were resected during the pancreaticoduodenectomy and reconnected using a polytetrafluoroethylene prosthesis. On day 1, early graft thrombosis was treated by thrombectomy and change to a larger graft. Pathology confirmed a R0-resection of the adenocarcinoma of the papilla of vater (pTis pN0,G2). At three-month follow-up, the patient was cancer-free and clinically asymptomatic, although, a late graft thrombosis with accompanying newly build venous collaterals passing mesenteric blood to the SV were found., Discussion: Rare PV disorders like a PDSMV do not contradict pancreatic surgery, but should be treated in experienced centres. Skills of SMV/PV reconstruction and its peri-operative management might be beneficial for successful outcome. Despite late graft thrombosis no clinical disadvantage occurred most likely due to preservation of the SV and of potential venous collateral pathways., Conclusion: Extended surgical procedures like a pancreaticoduodenectomy are realisable in patients with PV disorders, but require awareness, adequate radiological interpretation and specific surgical experience for secure treatment., (Copyright © 2015 The Authors. Published by Elsevier Ltd.. All rights reserved.)