A heterozygous dominantly acting likely pathogenic de novo WNT10A (NM_025216.3: c.283G > A; P.Glu95Lys) variant was found in a patient with isolated oligodontia, congenital absence of eight teeth, primary teeth retention, dental malocclusion (as well as shape anomalies), and a moderated lack of alveolar growth (Family 3). This evidence concerns the gene WNT10A and Oligodontia.