WNT10A and Oligodontia: 2012), despite the opposing supernumerary teeth phenotype observed in Wnt10a null mice (Yang et al. 2015; Xu et al. 2017). Here, we report the clinical and functional impact of a compound heterozygous mutation in WNT10A (c.637G>A (p.Gly213Ser); c.1070C>T (p.Thr357Ile) as likely pathogenic for isolated oligodontia, and demonstrate that perturbations in wnt10a expression impairs normal tooth development in zebrafish embryos.