Steele syndrome is a rare genetic bone disease caused by mutations in the COL27A1 gene, which encodes collagen type XXVII[4]. To date, more than 60 patients, most of whom are Puerto Rican, have been reported in the medical literature with the same p.Gly697Arg mutation, suggesting a common ancestor[1]. The article describes two new cases from the Russian and Uzbek populations with different mutations, expanding the knowledge of the genetic diversity of this disease[4]. Steele syndrome presents with characteristic bone changes including hip dysplasia, radial head dislocation, carpal fusion, and facial changes[2][8]. A study of 63 cases revealed that patients with certain types of mutations are more likely to have other manifestations outside the skeletal system and more severe short stature[4]. Research suggests that the type of mutation in the COL27A1 gene is related to different clinical manifestations of the disease, which may help in earlier diagnosis and selection of an appropriate treatment approach[4].