The management of metastatic renal cell carcinoma has undergone a revolution in the past year with groundbreaking treatment strategies encompassing a broad range of therapeutic modalities. At the other end of the spectrum, emerging data is beginning to change our perspective about the management of small, localized renal tumors that are being discovered with increasing frequency. This review will update recent findings supporting diet and tobacco exposure as etiologic factors in the development of renal cell carcinoma, the molecular concepts that underlie the disease and the targeted therapeutics designed to inhibit specific kinase activities, and emerging use of minimally invasive therapies for localized disease.