This systematic review aims to explore the intricate relationship between celiac disease (CD) and dental enamel defects (DED), focusing on pathophysiological mechanisms, oral health implications, and the role of dentists in early diagnosis. The review followed PRISMA guidelines and identified 153 publications from 2013 to 2024 across multiple databases. After exclusions, 18 studies met the criteria for qualitative analysis. Key findings include a high prevalence of DED in CD patients, ranging from 50% to 94.1%, with symmetrical and chronological defects being predominant. Significant associations were observed between CD severity and enamel defect extent. The review emphasizes the importance of early recognition of oral lesions, particularly through Aine’s classification, for potential CD diagnosis even without gastrointestinal symptoms. Collaboration between dentists and gastroenterologists is crucial for effective monitoring and management. The review consolidates current knowledge and highlights the need for further large-scale prospective research to deepen understanding and improve CD-related oral health outcomes.This systematic review aims to explore the intricate relationship between celiac disease (CD) and dental enamel defects (DED), focusing on pathophysiological mechanisms, oral health implications, and the role of dentists in early diagnosis. The review followed PRISMA guidelines and identified 153 publications from 2013 to 2024 across multiple databases. After exclusions, 18 studies met the criteria for qualitative analysis. Key findings include a high prevalence of DED in CD patients, ranging from 50% to 94.1%, with symmetrical and chronological defects being predominant. Significant associations were observed between CD severity and enamel defect extent. The review emphasizes the importance of early recognition of oral lesions, particularly through Aine’s classification, for potential CD diagnosis even without gastrointestinal symptoms. Collaboration between dentists and gastroenterologists is crucial for effective monitoring and management. The review consolidates current knowledge and highlights the need for further large-scale prospective research to deepen understanding and improve CD-related oral health outcomes.