While there was a trend toward increased risk with an increased number of variants, patients with three risky variants in BARD1 (rs7585356, rs6435862, and rs3768716) were significantly associated with nephroblastoma susceptibility with an odds ratio of 2.21, and subgroup analysis identified increased risk in females and development of stage I and II disease. Here, BARD1 is linked to Nephroblastoma.