Using a panel of 6 nonsynonymous SNPs of HELZ2 selected for association in 2 cohorts of Chinese Han, our results showed significant association of a rare SNP, rs79267778 with PBC in Han Chinese (ORcombined = 4.204 [1.670−10.582], pcombined = 1.87E−04). The gene discussed is HELZ2; the disease is primary biliary cholangitis.