BACKGROUND Survival of newborns with esophageal atresia and tracheoesophageal fistula has increased in the last years. OBJECTIVE To assess the prevalence of esophageal atresia and describe associated malformations in Chilean newborns. METHODS All births occurring between January 1983 and June 1998 were studied. All malformed children were registered and the next non malformed born child was considered as control. RESULTS During the study period, 50,965 births occurred and 3,336 malformed children were born. Eighteen (3 stillborn) had esophageal atresia with a rate of 3,53 per 10,000 born alive. Overall survival was 73%. Survival among children classified in Waterson groups A and B was 100% and 50% among those classified in group C. Seventy two percent had associated malformations, being congenital cardiopathies and skeletal malformations the most frequent. VACTERL association was found in 44% of children. All stillborn children had other severe malformations. When compared to controls, malformed children had a lower weight, a lower gestational age, their mothers had a higher age, a higher frequency of relatives with malformations and a higher frequency of maternal diseases during the first trimester of pregnancy. CONCLUSIONS The rate of esophageal atresia found in this study is similar to that reported in other Chilean obstetrical units as part of the Latin American Study of Congenital Malformations (ECLAMC).