Gonadoblastoma was reported in 4 patients, including 1 patient (aged 23 years) with an intron 9 splice-site variant who also had a dysgerminoma, 1 patient with a WT1 deletion at the age of 8 months, and 2 patients with missense variants in exon 9 (aged 1 and 3 years). Here, WT1 is linked to gonadoblastoma.