The International Consortium on MTC of 1996 (Eng et al. 1996), the 2001 Consensus on MENs (Brandi et al. 2001) and the updated American Thyroid Association (ATA) Consensus on MTC (Kloos et al. 2009) recommend that individuals carrying a germline disease-causing RET mutation should be subjected to early prophylactic total thyroidectomy, which is currently the only effective approach for preventing the progression of MTC. The gene discussed is RET; the disease is medullary thyroid gland carcinoma.