Background: Small bowel cancers, specifically duodenal cancer, occur at very low rates but require aggressive surgical resection when diagnosed. An even rarer finding is the presence of intestinal malrotation. Case Presentation: We present the unique case of a patient with both duodenal cancer and partial intestinal malrotation undergoing pancreaticoduodenectomy. We discuss the challenges faced and techniques used to successfully perform a surgical resection in this circumstance. Conclusion: Understanding of intestinal malrotation and review of the imaging is crucial in preparing for a resection of a duodenal tumor in a patient with this condition.