Malignant kidney tumors account for approximately 2% of all new primary cancer cases diagnosed in the United States, with an estimated 30,000 cases occurring annually. Although a variety of agents, chemical and biological, have been implicated as causal agents in the development of renal cell carcinoma (RCC), the etiology remains enigmatic. The strongest association has been developed between cigarette smoking and renal cancer however consistent, positive associations between RCC and obesity, diabetes, and hypertension have also been reported. In addition, more recent investigations of familial kidney cancer syndromes indicate that a strong genetic component contributes to RCC development. Several genes have been identified through investigation of familial kidney cancer syndromes. This review article describes recent trends in RCC incidence and the currently identifiable etiological causes that account for approximately half of the RCC cases diagnoses. The remainder of this review then focuses on additional risk factors that have thus far not been well examined but may be helpful in explaining the increasing incidence trends and the geographic or racial variation observed nationally and worldwide.