Renal cell carcinoma is most common in patients in the seventh decade of life. However, renal cell carcinoma (RCC) patients under the age of 40 represent 3-7% of all renal tumors. These young patients develop RCC from a variety of mechanisms including genetic syndromes, heritable mutations, and sporadic mutations. This population encompasses a distinct clinical entity that requires early identification and adjustments in standard practices including sometimes-aggressive surgical measures in order to improve oncologic management.