Introduction: We would like to present a rare case of metastatic renal tumor. Case presentation: A 60-year-old woman presented to our department with a left renal tumor. She underwent a total hysterectomy and right adnexal resection for a stage IA ovarian granulosa cell tumor approximately 15years ago, followed by left adnexal resection and postoperative chemotherapy with gemcitabine and paclitaxel 6years ago. She received six courses of gemcitabine and carboplatin to treat a stage IC clear cell adenocarcinoma of the ovary. The patient was diagnosed with the left renal tumor and underwent a laparoscopic left nephrectomy. Immunostaining was positive for alpha-inhibin and SF-1 and showed FOXL2 402C -> G (C134W) mutation. Finally, the patient was diagnosed with renal metastasis of a granulosa cell tumor. Conclusion: To our knowledge, this is a very rare case of renal metastasis of a granulosa cell tumor with the FOXL2 mutation in an adult.