A 50-year-old woman had undergone left nephrectomy for renal cell carcinoma 13 years previously. Ten years later, a solitary metastatic tumor had been detected in the pancreatic tail and she had undergone subsequent resection of the pancreatic tail and spleen. Three years after surgery, she was admitted to our hospital for severe anemia resulting from gastrointestinal tract bleeding. Esophagogastroduodenoscopy revealed a 3-cm solid tumor at the oral side of the papilla of Vater. Histology of the bioptic duodenal tissue revealed inflammatory granulation without malignancy. Computed tomography showed a well-contrasted hypervascular tumor in the descending portion of the duodenum. We diagnosed the patient with metachronous duodenal metastasis of renal cell carcinoma and performed a pancreaticoduodenectomy. An ulcerated polypoid mass was detected at the oral side of the papilla of Vater. Histology revealed clear cell carcinoma coated by granulation tissue across the surface of the tumor. Immunohistology demonstrated that the cells were positive for vimentin, CD10 and epithelial membrane antigen and negative for CK7. After a repeated pancreatectomy, the patient had no symptoms of gastrointestinal bleeding and maintained good glucose tolerance without insulin therapy because the remnant pancreas functioned well. In conclusion, for the diagnosis of patients who have previously undergone nephrectomy and present with gastrointestinal bleeding, the possibility of metastasis to the gastrointestinal tract, including the duodenum, should be considered. With respect to surgical treatment, the pancreas should be minimally resected to maintain a free surgical margin during the first surgery taking into account further metachronous metastasis to the duodenum and pancreas.