The variant was identified in five affected patients but not in the eight unaffected family members using Sanger sequencing and was classified as "pathogenic" according to the latest recommendation on sequence variants laid out by the American College of Medical Genetics and Genomics and the Association for Molecular Pathology.<h4>Conclusion</h4>We described a family with CHD caused by a novel TAB2 nonsense mutation. Here, TAB2 is linked to coronary artery disorder.