Renal cell carcinoma in adult comprises a heterogeneous group of tumors with variable clinical outcomes, which ranges from indolent to aggressively malignant. The application of molecular genetics techniques to the study of renal neoplasms has resulted in improved classification of these entities and better understanding of biological mechanisms responsible for tumor development and progression. In the present article we review the molecular genetic profiles of different renal cell tumors and discuss their relevance to the carcinogenesis mechanisms and to the clinical diagnosis of renal cell carcinoma. Understanding of the molecular genetics of renal tumors is beneficial in making accurate diagnoses, assessing prognoses, and selecting appropriate and targeted therapeutic options.