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PURPOSE: to evaluate associations between parental exposure to pesticides and births with congenital defects in São Francisco Valley, as well as the demographic profile and the defects found. METHODS: in this case-control study, each case (newborns with congenital defects) had two controls (healthy newborns). The subjects were born in the city of Petrolina, in São Francisco Valley, in 2009. The sample consisted of 42 cases and 84 controls. Data were gathered by a structured questionnaire adapted from Latin-American Collaborative Study of Congenital Malformations (ECLAMC), with the addition of questions related to exposure to pesticides, analysis of the medical records and contact with the hospital's pediatrician. The χ2 test was performed with a significance level of 5% to identify the variables with the greatest differences between case and control groups. Odds Ratio (OR) for the sample was calculated, as well as the OR obtained by logistic regression analysis, and finally, multivariate logistic regression analysis was performed. RESULTS: there was a greater exposure to pesticides during pregnancy in infants with congenital defects compared to healthy subjects. Increased risk was observed when at least one parent was exposed to pesticides (adjusted OR = 1.3; 95%CI = 0.4 - 3.9). The sociodemographic variables associated with congenital defects were: low school level, low weight, prematurity, young parents, chronic diseases, and physical factors. Multiple malformations and defects of the musculoskeletal and nervous systems were more frequently found. CONCLUSIONS: the present study suggests an association between exposure to pesticides and the occurrence of congenital defects, although the data were not significant.