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Introduction We report a rare case of renal cell carcinoma with vaginal metastasis that recurred with rapid progression and was resistant to sunitinib and nivolumab. Case presentation A 68‐year‐old woman presented with renal cell carcinoma and vaginal metastasis. Multiple lung metastasis appeared 3 months after simultaneous radical nephrectomy and hysterectomy with vaginal resection. Despite the treatment with sunitinib and nivolumab, the patient died 7 months after surgery. Immunohistochemical staining of primary and metastatic tumor specimens was CD8 and programmed death ligand‐1 negative. Conclusion Although vaginal metastasis of renal cell carcinoma is rare, lack of CD8 and programmed death ligand‐1 expression may cause nivolumab resistance and may be useful markers in patients with metastatic renal cell carcinoma.