We have demonstrated apodictically that Steel syndrome is an AR disorder clinically characterized by congenital bilateral hip dislocation, short stature, scoliosis, radial head dislocation, carpal coalitions, and foot deformities; and molecularly defined by homozygosity for the c.2089G>C (p.Gly697Arg) variant in COL27A1. The reported allelic series in COL27A1 provides further insights into disease biology and genetic contributions to clinical phenotypes in these COL27opathies. This evidence concerns the gene COL27A1 and Steel syndrome.