Carvalho P. Epidemiological study of developmental enamel defects in children aged 2 to 4 years in the municipality of Santa Isabel, SP. [thesis]. São Paulo: Universidade de São Paulo, Faculdade de Odontologia; 2020. Versão Corrigida. The disorders that occur during enamel formation of primary teeth that manifest as Developmental Enamel Defects (DDE) have an important clinical significance, as they are responsible for aesthetic problems, occlusion disorders, dentin sensitivity and they can act as a predisposing factor to tooth decay and a predictor of molar incisor hypomineralization. These possible clinical implications can negatively impact the oral health-related quality of life (OHRQoL) of children with DDE. The aim of the present study was to know the prevalence and severity of DDE in primary teeth, to identify the etiological factors associated with the defects of enamel and to assess the impact of DDE in OHRQoL. The study evaluated 336 children aged 2 to 4 years in the municipality of Santa Isabel, São Paulo who attended the National Day of Children Vaccination. For the diagnosis of DDE, the extent, location, color of the defect were evaluated and the type of defect was also determined using the modified DDE index recommended by the International Dental Federation. Mothers or legal caregivers answered the Brazilian version of the Early Childhood Oral Health Impact Scale (B-ECOHIS), socio-demographic and the pre, peri and post-natal conditions questionnaires. To assess the impact of DDE on OHRQoL, data were collected using the Brazilian version of the ECOHIS questionnaire. Descriptive analyzes, Kappa test, chi-square test, Kolgomorov Smirnov normality test, Wilcoxon test, unadjusted and adjusted Poisson regression analyzes (α = 0.05) were performed. The prevalence of DDE was 50.60%. Demarcated (45.0%) and diffuse (36.0%) opacities of white / cream color were the most frequent defects. Molars were the teeth most affected, and among the surfaces examined, the buccal surfaces were the most affected. There was an association between DDE and alcohol consumption during pregnancy (p = 0.022), hospitalization of the child in the first year of life for infectious diseases (p = 0.010) and dental caries (p = 0.022). Children who were breastfed for 12 months had a lower risk of developing DDE (p = 0.022). Yellow-brown opacities were more likely to have a negative impact on the symptom and limitation domains (p <0.05) and on the parents' distress domain (p <0.05). It can be concluded that DDE have high prevalence and mild severity. Alcohol consumption during pregnancy and the child's hospitalization for infectious diseases in the first year of life are risk factors for DDE in primary dentition. Breastfeeding for a period of 12 months is a protective factor for the development of DDE in the primary dentition. The demarcated yellow-brown opacities have an negative impact on the OHRQoL of children aged 2 to 4 years, according to the parents' reports.