Pathogenic WNT10A (MIM *606268) variants can be responsible for isolated tooth agenesis as well as for odonto-onycho-dermal dysplasia (OODD; MIM #257980) and Schöpf–Schulz–Passarge syndrome (SSPS; MIM #224750). Here, WNT10A is linked to odonto-onycho-dermal dysplasia.