Ovarian metastases from renal cell carcinoma are rare, with only 22 cases reported in the literature. We report a case of a 45-year-old woman, who developed left ovarian and right adrenal metastases 3 months after diagnosis of clear cell renal cell carcinoma and review the literature. This is the fourth reported case of right renal cell carcinoma metastasizing to the left ovary. The patient is alive 4 years after resection of the ovarian tumor, treated with sunitinib. We conclude that, although rare, metastatic renal cell carcinoma should be included in the differential diagnosis of ovarian tumors with clear cell histology.