This paper discusses a rare case of renal metastatic osteosarcoma. A 25-year-old man with a history of metastatic osteosarcoma involving his right kidney was referred to our institution for treatment. He was managed with chemotherapy. An exhaustive review of the English literature pertaining to this disease was performed. To our knowledge, this case represents only the sixteenth. The literature suggests that the incidence of renal involvement in osteosarcoma is significant and that the treatment should be multidisciplinary in such patients.