Renal cell carcinoma (RCC) is uncommon in adolescents and young adults, although rare variants of the disease, including Xp11.2 translocation carcinoma, collecting duct carcinoma, and renal medullary carcinoma, occur with a higher preponderance in young patients. Likewise, non-RCC kidney tumors such as those of the Ewing sarcoma family of tumors are also seen in younger patients. The mainstay therapy for most forms of localized RCC and other kidney cancer is surgical removal of the tumors. Advances in treatments for metastatic clear cell carcinoma have been made in the last decade with the development of several new targeted agents. These therapies have revolutionized the treatment of metastatic clear cell RCC but are not targeted for other types of kidney cancer that are more often found in young patients. The management of RCC in adolescents and young adults remains a challenge for clinicians, but further advances are anticipated as less selective targeted immunotherapies become more widely available.