Kyphoscoliotic type of Ehlers–Danlo syndrome occurs in the practice of doctors of different specialties. The main manifestations of the disease are congenital muscular hypotonia, progressive kyphoscoliosis, generalized joint hypermobility, dislocation/subluxation of the joints. In recent years, special attention has been paid to the fragility of blood vessels characteristic of patients, which usually has serious consequences. Purpose: the study aims at clarifying the clinical characteristics of type 1 kyphoscoliotic Ehlers–Danlo syndrome based on the analysis of the clinical findings observed in 2 patients with severe vascular disorders. Data from clinical and molecular genetic examination of 2 children (girl and boy) 17 and 13 years old from unrelated families are presented. The diagnosis of type 1 kyphoscoliotic Ehlers–Danlo syndrome was established in accordance with the diagnostic criteria according to the International Classification 2017. Children showed severe vascular disorders in the neonatal period, ruptures of medium-caliber arteries in adolescence. Genomic sequencing in both patients revealed a pathogenic nucleotide variant in the PLOD1 gene — duplication of the 11959421–11968422 chromosome 1 region in the homozygous state. The results of literature analysis and own observations of 2 patients with type 1 kyphoscoliotic Ehlers–Danlo syndrome indicate the severity and rather high incidence of vascular disorders, which should be considered when developing recommendations for the medical management of patients.