In conclusion, our study identified a missense variant (NM_014244: c.3506G>T) in exon 22 of ADAMTS2 associated with susceptibility to skeletal Class III malocclusion and maxillary deficiency and proved that ADAMTS2 may be related to craniofacial development in vitro and in vivo. Here, ADAMTS2 is linked to Hypoplasia of the maxilla.