Objectives: Since Spitz et al. reported the prognostic classification of esophageal atresia (EA) patients in 1994, decades have been past and there have been many advances in surgery and neonatology. Nevertheless, there have been very few reports according to the recent outcome of the neonates with EA, and otherwise, time has come to re-evaluate the credibility of this classification. The aim of this study was to validate the recent prognosis of the EA. Methods: Patient data were collected from 22 cooperative facilities during the 5 year period from 2005 to 2009 in Kyushu area, Japan. Total of 100 EA patients were retrospectively reviewed according to their characteristics and the outcome. Patient who missed the characteristics and outcome was excluded from the respective data. Results: Only 29.8% (28/94) was prenatally diagnosed and 52.0 (52/100) had associated anomalies including major congenital heart disease (CHD), abnormal chromosome, and others. According to the operation, primary anastomosis was performed 57.0% (57/100) and the staged operation was performed 34.0% (34/100). Survival rate in the neonatal period was 89.0% (89/ 100), and overall survival rate was 78.0% (78/100). According to the Spitz classification, if patients with associated anomalies were excluded, survival rate of Group 1 (>1500 g and no CHD) was 93.8% (61/65), Group 2 (<1500 g or CHD) was 68.4% (13/19), and Group 3 (<1500 g and CHD) was 50% (1/2). Conclusion: EA was proved to be rarely diagnosed prenatally. Primary outcome of the Group 1 and Group 3 in Spitz classification were fairly good, but Group 2 was worse as ever. The comprehensive treatment strategy for EA patients with birth weight under 1500 g or CHD should be reconsidered to improve the overall outcome.