AbstractAimCoeliac disease can induce specific enamel defects (SED), but little is known about the consequences of antitissue transglutaminase (TG2A) autoimmunity. We investigated whether TG2A positivity in children and their mothers was associated with SED in the primary dentition.MethodsMaternal and child serum immunoglobulin A‐TG2A levels were measured as part of the Generation R prospective cohort study. Clinical oral photographs of the primary dentition were taken, and SED and caries were recorded. We performed logistic regression analysis.ResultsWe analysed data on 4775 mothers and 4233 children (median age of 6.2 ± 0.5 years). SED and caries were not associated with maternal TG2A levels. The 59 TG2A‐positive children tended to have more SED, particularly the 31 in the strongly positive subgroup, with odds ratio of 1.72 and 2.29, respectively. A positive linear trend was observed between higher TG2A levels and paediatric SED (p = 0.04), but this became nonsignificant after adjusting for ethnic and socio‐economic background. No difference in caries was found between the groups.ConclusionTG2A did not play an independent role on SED in the primary dentition during pregnancy and childhood, and the relationship may be explained by ethnic and socio‐economic background.