In the 25 individuals with a DICER1 pLOF variant compared with 7550 noncarriers matched by age, sex, race, and smoking status, we observed significantly greater associations with thyroid cancer (OR, 9.2; 95% CI, 2.1-34.7; P = .02) and thyroidectomy (OR, 6.0; 95% CI, 2.2-16.3; P = .007); thyroidectomy remained significant after Bonferroni correction (Figure 1; eTable 5 in the Supplement). Here, DICER1 is linked to thyroid gland carcinoma.