BACKGROUND: Molar incisor hypomineralization (MIH) is a developmental enamel defect with multifactorial etiology. Although the relationship between celiac disease (CD) and developmental enamel defect was demonstrated, the association between CD and MIH is uncertain. OBJECTIVE: The objective of this study was to analyze the occurrence of MIH in CD patients. METHODS: Forty CD patients and a control group with 40 healthy individuals were selected. A calibrated examiner (k≥0.889) according to the European Academy of Pediatric Dentistry criteria performed the diagnosis of MIH. Data were analyzed by descriptive statistics and Fischer’s exact test (α=0.05). RESULTS: Of the 80 participants, ten presented MIH with eight individuals with CD. Celiac patients presented 4.75 times the chance of occurrence of MIH than the control group (95% CI: 2.22-10.18; P=0.044). In all the evaluated teeth (n=978), 22 had MIH: 20 teeth in individuals with CD and two in those without the disease. All CD participants with MIH presented the classic form of the disease. CD participants showed 17 teeth (85.0%) with demarcated opacities, two (10.0%) post-eruptive collapses and one (5.0%) atypical restoration. The control group presented only demarcated opacities. CONCLUSION: CD increased the chance of MIH and associated with its clinical manifestations can assist in the diagnosis of CD.