We report the first case of duodenal-inferior vena caval (IVC) fistula resulting from locally recurrent renal cell carcinoma (RCC). A 45-year-old man presented with gross hematuria and underwent a right radical nephrectomy to treat a solid renal mass. Histologic evaluation showed RCC, State pT3aNOMO. The patient presented 21 months later in hemorrhagic shock, with upper gastrointestinal bleeding. He underwent an exploratory laparatomy and Whipple procedure for a mass in the second portion of the duodenum extending to the inferior vena cava with a secondary duodenal-IVC fistula. We describe this case and review the previously published reports of duodenal-IVC fistulae.