For instance, DI may be linked to mutations in KIF4A and ADAR1.43,44 Taurodontism is commonly associated with hereditary systemic diseases,37 such as odonto-onycho-dermal dysplasia caused by WNT10A mutation.45 Recent studies have elucidated the causal relationship between Wnt10a mutation and taurodontism.46,47 However, taurodontism can also occur as an isolated condition without systemic manifestations.48 The pathogenic mechanisms underlying non-syndromic taurodontism, however, remain largely unknown. Here, ADAR is linked to taurodontism.