Since 2011, the MITF gene (Microphtalmia-Associated Transcription Factor) is considered as a medium-risk gene, since two studies involving linkage analysis followed by sequencing, showed the functional impact of the E318K rare mutation on melanoma and renal cell carcinoma risks (Bertolotto et al., 2011; Yokoyama et al., 2011). This evidence concerns the gene MITF and hereditary clear cell renal cell carcinoma.