The proband (#660 II-1) with a biallelic pathogenic WNT10A variant (c.1176C > A; p.Cys392* and c.812G > A; p.Cys271Tyr) exhibited severe syndromic tooth agenesis with classical OODD features, characterized by agenesis of all permanent teeth, hyperkeratotic palms and soles, dystrophic toenails and fingernails, and sparse hair. Here, WNT10A is linked to tooth agenesis.