Recent studies of large CAH cohorts in different continents revealed that approximately 15% of the CAH population has hEDS due to a contiguous gene deletion affecting both the CYP21A2 and TNXB genes [10,11,12,13], which warrants re-examination of current CAH clinical and genetic evaluation workflows [14,15,16]. This evidence concerns the gene TNXB and congenital adrenal hyperplasia.