Renal cell carcinoma is the most common primary renal neoplasm in adults. Although renal cell carcinoma is known to spread to unusual sites, the ampulla of Vater is considered a rare site for metastasis. Here we present a case of renal cell carcinoma metastasized to the ampulla of Vater along with literature review. A 62-year-old Korean male had a history of hypertension and right-sided renal cell carcinoma diagnosed in September 2004, for which he underwent right radical nephrectomy in October 2004. The patient eventually underwent laparoscopic pylorus-preserving total pancreaticoduodenectomy in January 2017. The surgery was successful without postoperative complications. Previous studies have shown that surgical resection for solitary metastases of renal cell carcinoma can provide favorable survival rates. Our case report provides evidence that pancreaticoduodenectomy may be a treatment of choice for suitable patients with solitary renal cell carcinoma ampullary metastasis. A minimally invasive approach may result in early recovery of patient to be suitable for subsequent chemotherapy. Further evidence is needed to address the exact role of minimally invasive pancreaticoduodenectomy in renal cell carcinoma metastasized to the ampulla of Vater.