As its name implies, TNX-deficiency or classical-like EDS (clEDS) phenotypically resembles the classical form of EDS (cEDS), with the triad of soft/velvety hyperextensible skin (20/20), generalized joint hypermobility (15/19) and a varying degree of tissue fragility (20/20) as its main clinical features (Table 1) [9]. Here, TNXB is linked to Ehlers-Danlos syndrome.