The identification of a novel de novo missense variant, c.1231G>T (p.Asp411Tyr) and known missense variant, c.1252T>C (p.Tyr418His) in LRP6 within Thai individuals diagnosed with oligodontia substantially reinforces the indispensable role of LRP6 in orchestrating human dental development. Here, LRP6 is linked to Oligodontia.