Objectives: Studies have observed the presence of extra-intestinal manifestations of celiac disease (CD), including involvement of the oral cavity, such that developmental defects of enamel (DDE) occur. Thus, the aim of this review was to access the polled prevalence of DDE in individuals with CD, and to establish the strength of the association between these two variables. Methods: To carry out the systematic review, four electronic databases and the Grey Literature were searched, complemented by a manual search of reference lists within the selected articles. Two pairs of independent reviewers selected the articles, and perform the data extractions and bias risk assessment Studies evaluating the presence of DDE in individuals with CD as well as in healthy individuals and which performed the DDE diagnosis by direct visualization of tooth enamel changes and the CD diagnosis were included. Meta-analyses were performed using the software R. Results: Of 557 studies, 45 were selected for review, encompassing 2840 patients. The prevalence of DDE in people with CD was 50% (95% CI 0.44–0.57, I2 = 88%). In a general analysis, it was observed that patients with CD had a significantly higher prevalence of enamel defects compared to healthy people (RR: 2.31, 95% CI: 1.71–3.12, I2 = 98%). Only developmental defects of enamel diagnosed using Aine’s method were associated with the disease (RR: 3.30, 95% CI 2.39–4.56, I2 = 75%). In a sensitivity analysis involving the deciduous, mixed and permanent dentitions, only individuals with deciduous dentition were observed to have association with the disease (RR: 2.34, 95% CI 1.25–4.39, I2 = 39%). Conclusions: Patients with enamel developmental defects should be screened for the possibility of their having celiac disease.