Perineal involvement in renal cell carcinoma (RCC) has not been reported. Vaginal metastases of RCC are also rare. We present a case of metastatic RCC, initially presenting as a perineal cyst. A 53-year-old woman presented with a perineal cyst, which was excised and diagnosed as clear cell carcinoma. A workup further revealed a vaginal mass and a renal tumor. A nephrectomy and local vaginal excision were performed, leading to the diagnosis of metastatic RCC. This case illustrates the variability in RCC presentation. Also, because vaginal clear cell carcinoma is rare, all such lesions should be considered potentially renal in origin.