We reviewed the current knowledge of the role of a genetic variant, 1100delC, of CHEK2 in prostate cancer risk and found pooled odds ratios of prostate cancer for CHEK2∗1100delC heterozygote of 1.98 (95% CI: 1.23–3.18) for unselected cases and 3.39 (95% CI: 1.78–6.47) for familial cases versus noncarriers, suggesting that screening for CHEK2∗1100delC should be considered in men with a familial history of prostate cancer. The gene discussed is CHEK2; the disease is Familial prostate cancer.