Familial and syndromic Wilms tumours have demonstrated the susceptibility of the developing kidney to germline variants of WT1 in children with genitourinary abnormalities [14], BRCA2 and PALB2 in Fanconi anaemia patients [15, 16], GPC3 in Simpson-Golabi-Behmel syndrome patients [17], DIS3L2 in Perlman syndrome [18], DICER1 in DICER1-related disease [19], BUB1B and TRIP13 in mosaic variegated aneuploidy (MVA) syndrome [20, 21], and CTR9 [22], REST [23], PALB2, and CHEK2 [13] in non-syndromic Wilms tumour families. The gene discussed is DICER1; the disease is mosaic variegated aneuploidy syndrome 1.