In addition to the role of Tuberin in the benign tumours of TSC patients, Tuberin mutations have also been found to cooperate with oncogenic mutations aiding in the initiation and progression of a number of malignant cancers affecting the brain (medulloblastoma), lung, kidney (renal cell carcinomas), and breast (Dabora et al., 2001; O’Callaghan et al., 2004; Bhatia et al., 2009; Franz et al., 2010; Orlova and Crino, 2010). Here, TSC2 is linked to hereditary clear cell renal cell carcinoma.