The case here reported is comparable to previous reported ones, when initially there is suspicion of EDS on clinical ground but molecular testing for COL5A1 or COL5A2 (related to classical EDS) variants are negative and, oddly, variants in COL1A1 and COL1A2, primarily ascribed as causative of OI, are confirmed (Shi et al., 2015; Malfait et al., 2017; Morlino et al., 2020). This evidence concerns the gene COL5A2 and Ehlers-Danlos syndrome.