We have identified a rare nonsense variant (c.1282C > T/p.Arg428*) in ZNF528 in patients who were previously reported to have a deletion in COL1A2 and suffered from severe early onset primary osteoporosis but lacked the typical features of either OI or EDS.(32) The biological function of ZNF528 is poorly understood and it has not previously been associated with bone or connective tissue‐related phenotypes. Here, ZNF528 is linked to Ehlers-Danlos syndrome.