The aim of this study was to investigate the presence and distribution of developmental enamel defects and caries in children with celiac disease (CD) and compare the results obtained with those of a control group of children without CD. A total of 64 subjects (mean age 8.2 years) selected from patients diagnosed and treated for CD were studied. Sixty-four age/ sex-matched healthy children were enrolled as a control group. Permanent dentition enamel defects were recorded according to Aine's classification. The caries experience of the children was recorded according to the criteria of the World Health Organization (WHO). The prevalence of enamel defect in CD subjects was found to be significantly higher (42.2%) than in healthy subjects (9.4%) (p < 0.001). Grade I type enamel defects were most commonly diagnosed in both groups (20.3% and 6.3%, respectively). The number of caries-free subjects in the control group was higher (38%) than in the CD group (17%). This study clearly showed that children with CD were at an increased risk of dental enamel defects compared with healthy subjects. Enamel defects were associated with an increased caries incidence.