Purpose: The purpose of this study was to find potential risk factors associated with the occurrence of developmental defects of enamel (DDE) in permanent dentition of a birth cohort in Southern Brazil. Methods: This study is a 2004 birth cohort carried out in the city of Pelotas, Rio Grande do Sul, Brazil. A subsample of 996 children was clinically examined at 12 to 13 years of age. The pre- and perinatal variables used for this study were: twin pregnancy; type of delivery; prematurity; birth weight; Apgar score in the first and fifth minute; health problems at birth; and intensive care unit admission at birth. Gender and family income variables were also collected. Some postnatal variables were collected: "hospitalization in the first 24 months"; "breastfeeding"; "respiratory diseases in the first 48 months"; "urinary infection in the first 48 months"; and "ear pain in the first 48 months." To assess an association between the presence of DDE and various risk factors, unadjusted and adjusted Poisson regression with robust variance was performed with a respective prevalence ratio. Results: The prevalence of DDE was 40.8 percent. The variables investigated did not present a statistical association with DDE. Conclusion: No association of developmental defects of enamel in permanent dentition with any of the factors investigated in this study was found.