Kidney cancer accounts for approximately 3% of all adult malignancies, and life-long incidence is 1/72, thus it is not a rare entity. Kidney cancer occurs in both sporadic and hereditary forms. Conservative estimates have suggested that 3-5% of renal cancer patients have inherited forms of disease. Research of these syndromes and, especially in last decade, discovery of the genes responsible for heritable forms of kidney cancer and their association with similar sporadically occurring tumors has helped to shed light on the molecular pathways of tumorigenesis and the potential for targeted molecular therapy. In clear cell renal cell carcinoma, sunitinib, sorafenib and temsirolimus, molecules that target Von Hippel Lindau gene pathway, are pioneer drugs of this aim. Advances in these treatment modalities, will let us new aspects in treatment of both sporadic and hereditary kidney cancer. Surgical interventions for each syndrome are also mentioned in this review.