Renal cell carcinomas account for 80-85% of all primary renal neoplasms. Recent identification ofVHL,c-metandTSCas candidate genes mutated in various types of renal carcinomas has greatly enhanced our understanding of the pathogenesis of renal carcinomas and has provided novel therapeutic options for patients with renal cancer. Furthermore, developments in angiogenesis and in tumor immunology have given us additional treatment modalities for cancer patients, especially those with renal cancer. This review highlights the genetic abnormalities seen in renal cell carcinomas and reviews current and future therapeutic options.