Renal cell carcinoma (RCC), the fifth leading malignant condition for menand tenth for women, accounts for 3% of all malignancies in Canada. It is aheterogeneous epithelial malignancy with different subtypes and varied tumourbiology. Although most cases of RCC are sporadic, up to 4% of patients havean inherited predisposition for the disease. In this article, we review the currentmolecular genetics of the different subtypes in hereditary and sporadic RCC. Significant developments in

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... g the underlying genetic basis of RCC over the last 2 decades are attributed to intensive research about rareinherited renal cancer syndromes and the identification of the genes responsiblefor them. Many of these genes are also found in sporadic RCC. Understanding the molecular mechanisms involved in the pathogenesis of RCC has aided the development of molecular-targeted drugs for this disease.