For instance, individuals with WT1 mutations have an enormous risk of Wilms tumor.7, 8 A study by Ciceri et al suggested that constitutional anomalies of CHEK2 play an important role in the development of Wilms tumor.9 Moreover, a series of genetic alterations were identified associate with Wilms tumor susceptibility, including WTX,10CITED1,11SIX1,12SIX2,13TP53,14, 15HACE1,16LIN28B,17 and KRAS. 18 Nevertheless, these genetic variations are not sufficient to explain the whole genetic pathogenesis involved in Wilms tumor. The gene discussed is CHEK2; the disease is Nephroblastoma.