By studying the population in northern Iran, where there is a considerable risk of developing ESCC, our team previously reported the BRCA2 p.K3326* mutation in 27 of 746 ESCC cases and in 16 of 1,373 controls (OR = 3.64, 95% CI = 1.74–7.59, P = 0.0003) [26]. Here, BRCA2 is linked to esophageal squamous cell carcinoma.