In this study, exome sequencing and Sanger sequencing detected the compound heterozygous variants, c.3426-1G>A and c.4306C>T (p.(Arg1436Trp)), in the dynein axonemal heavy chain 11 gene (DNAH11, NM_001277115.1) in a Han Chinese family with HTX and CHD. This evidence concerns the gene DNAH11 and coronary artery disorder.