Knowledge about renal cell carcinoma (RCC) has increased exponentially over the last decades. A clear understanding of RCC is of utmost importance to prevent the disease and improve the outcomes. Large epidemiologic studies have identified cigarette smoking, chemical agents, obesity, hypertension, and end-stage renal disease as risk factors associated with RCC. Identification and confirmation of risk factors may be projected into preventive strategies. Genetic studies of inherited disorders associated with an enhanced risk of RCC have elucidated many important targets for anticancer therapy. The World Health Organization (WHO) has recently developed a new histologic classification of renal cell tumors that has demonstrated prognostic utility. A refined clinical staging system is improving our ability to prognosticate the outcome of RCC patients. This article provides a practical yet comprehensive review of the risk factors, classification, and staging of RCC focusing on recent updates.