Kyphoscoliotic EDS demonstrates autosomal recessive inheritance and results from either deficiency of lysyl hydroxylase 1 due to mutations in PLOD1 or alterations in FKBP14, which encodes a peptidyl-prolyl cis–trans isomerase that functions as a molecular chaperone for multiple collagen types (III, IV, VI, and X) [9]. The gene discussed is PLOD1; the disease is Ehlers-Danlos syndrome.