Greater than 90% of ccRCC cases are characterized by loss of heterozygosity on the short arm of chromosome 3.[7] About 50% of the cases have a genetic mutations[7,8] while 5% to 10% of the cases are characterized by promoter hypermethylation.[8] These alterations lead to high-frequency inactivation of the biallelic VHL. Here, VHL is linked to nonpapillary renal cell carcinoma.