A systematic review was conducted to explore the relationship between celiac disease (CD) and dental enamel defects (DED). The review analyzed 153 publications, with 18 studies meeting the inclusion criteria. The findings indicate a high prevalence of DED in CD patients, ranging from 50 to 94.1%. Symmetrical and chronological defects, according to Aine's classification, were predominant, and significant associations were observed between CD severity and enamel defect extent. The review highlights the importance of early diagnosis of DEDs as potential indicators of CD, even in the absence of gastrointestinal symptoms. Correlations between CD and dental health conditions like molar incisor hypomineralization (MIH) emphasize the dentist's crucial role in early diagnosis. Collaboration between dentists and gastroenterologists is essential for effective monitoring and management. The review consolidates current knowledge, laying the groundwork for future research and promoting interdisciplinary collaboration for improved CD-related oral health outcomes. Further large-scale prospective research is recommended to deepen our understanding of these issues. The study also found that CD is associated with an increased risk of MIH, and early recognition of MIH could play an important role in the early diagnosis of CD. The review underscores the importance of involving dentists in the management and awareness of CD, especially in newly diagnosed patients. The study highlights the need for standardized diagnostic approaches and prospective studies to establish clearer causal links and strengthen the evidence base. The review also acknowledges limitations, including a reliance on retrospective studies, lack of consistency in sample sizes, and absence of standardized diagnostic criteria. Despite these challenges, the current evidence underscores the intricate link between CD and dental health, advocating for further extensive prospective studies to solidify and expand our understanding of this relationship.A systematic review was conducted to explore the relationship between celiac disease (CD) and dental enamel defects (DED). The review analyzed 153 publications, with 18 studies meeting the inclusion criteria. The findings indicate a high prevalence of DED in CD patients, ranging from 50 to 94.1%. Symmetrical and chronological defects, according to Aine's classification, were predominant, and significant associations were observed between CD severity and enamel defect extent. The review highlights the importance of early diagnosis of DEDs as potential indicators of CD, even in the absence of gastrointestinal symptoms. Correlations between CD and dental health conditions like molar incisor hypomineralization (MIH) emphasize the dentist's crucial role in early diagnosis. Collaboration between dentists and gastroenterologists is essential for effective monitoring and management. The review consolidates current knowledge, laying the groundwork for future research and promoting interdisciplinary collaboration for improved CD-related oral health outcomes. Further large-scale prospective research is recommended to deepen our understanding of these issues. The study also found that CD is associated with an increased risk of MIH, and early recognition of MIH could play an important role in the early diagnosis of CD. The review underscores the importance of involving dentists in the management and awareness of CD, especially in newly diagnosed patients. The study highlights the need for standardized diagnostic approaches and prospective studies to establish clearer causal links and strengthen the evidence base. The review also acknowledges limitations, including a reliance on retrospective studies, lack of consistency in sample sizes, and absence of standardized diagnostic criteria. Despite these challenges, the current evidence underscores the intricate link between CD and dental health, advocating for further extensive prospective studies to solidify and expand our understanding of this relationship.