Wilms tumor exhibits ahigh degree of genetic heterogeneity, and the related genes includeWT1 (chromosome 11p13), WTX (chromosomeXq11.1), CTNMB1 (chromosome 3p22.1) and TP53(chromosome 17p13.1) (Scott et al., 2006).Spreafico et al. reported a girl with a TSC2mutation who developed a unilateral Wilms tumor. Here, AMER1 is linked to Nephroblastoma.