Background: Celiac disease (CD) is an autoimmune disorder that harms the epithelium and lamina propria of the small intestine in genetically predisposed individuals. CD negatively affects oral and dental health. Objectives: This research explored the DMFT index and enamel defects (ED), as well as their association with oral health-related quality of life in children with CD. Methods: In this descriptive-analytical cross-sectional study, 50 children with CD (case group) and 50 healthy children (control group) were examined. The World Health Organization criteria were used to determine the DMFT index. Aine's classification was employed to diagnose ED, and the standard CHILD-Oral Impacts on Daily Performances scale was used to assess child oral health-related quality of life (COHRQoL). The Chi-Square test and Mann-Whitney test were utilized for data comparison. P-values < 0.05 were considered statistically significant. Results: The average DMFT was 2.58 ± 2.39 in the case group and 2.08 ± 1.61 in the control group. According to the Mann-Whitney U test, this difference was not statistically significant (P = .223). The frequency of ED in the case group was significantly higher at 52%, compared to 16% in the control group (P < .001). Additionally, there was a significant difference in the frequency of ED between boys and girls in the two groups (P < .001). The COHRQoL score in the case group, with an average of 24.44 ± 19.91, was significantly higher than that in the control group, which had an average of 13.35 ± 13.65 (P = .002). In both the case and control groups, a significant positive relationship was observed between the COHRQoL score in children and the DMFT index (P < .001). There was also a significant relationship between the COHRQoL score in children with ED in both the case and control groups (P = 0.004). Conclusions: According to the results, there was no difference in the DMFT index between the 2 groups. However, as the DMFT index increased in both groups, the COHRQoL score also increased. The number of ED cases was higher in celiac patients, and this had a significant effect on the COHRQoL score. Additionally, the COHRQoL score was higher in celiac patients compared to the control group.