This video shows the performance of an esophagectomy in a patient with polysplenia and discusses the special anatomic features which influence the choice of incision, extent of resection, and options for reconstruction in such patients. Absence of the suprarenal IVC with azygous continuation, pre-duodenal portal vein, and visceral vascu-lar and rotational abnormalities are the principal confounding fac-tors. Major complications could arise from failure to recognize and understand this syndrome.