Background: Celiac disease (CD) is a chronic immune-mediated gluten-sensitive enteropathy, affecting about 1% of the population. The most common symptoms include diarrhea, abdominal pain, weight loss, and malabsorption. Extra-intestinal symptoms include oral manifestations. This systematic review aims to catalog and characterize oral manifestations in patients with CD. Methods: a systematic literature review among different search engines using PICOS criteria has been performed. The studies included used the following criteria: tissues and anatomical structures of the oral cavity in humans, published in English and available in full text. Review articles and papers published before 1990 were excluded. Results: 209 articles were identified in the initial search. In the end, 33 articles met the selection criteria. The information extracted from the articles was classified based on the type of oral manifestation. Recurrent aphthous stomatitis (34.6%), atrophic glossitis and geographic tongue (15.26%), enamel defects (42.47%), delayed dental eruption (47.34%), xerostomia (38.05%), glossodynia (14.38%), and other manifestations including cheilitis, fissured tongue, periodontal diseases, and oral lichen planus were found in the celiac subjects of the studies analyzed. The quality of articles on the topic should be improved; however, oral manifestations in CD patients are widely described in the literature and could help diagnose celiac disease.