Objective: Oesophageal atresia is an congenital anomali with incidence of 1/3000-1/4500 live births. The results of reconstructive surgery has improved greatly, to 80-92% survival reported in the last two decades. The aim of this study was to determine the incidence of oesophageal atresia in Iceland and to evaluate the results of operations at the Department of Pediatric surgery at The Children's Hospital, Landspítal inn - University Hospital in Iceland. Material and methods: This retrospective study included all children diagnosed with oesophageal atresia in Iceland between 1963 and 2002. Information was gathered from hospital records, including birth-weight, gestational age, the type of atresia and the presence of other congenital anomalies. The results of operation were determined including post operative complications. Information on life births in Iceland for the same period was gathered from the Icelandic National Register. Results: Thirtyseven children were diagnosed with oesophageal atresia in these 40 years. The average birth-weight was 2626g, including 14 children (38%) with low birth-weight (<2500g). Fifteen children (41%) were prematurely born (<38 weeks). Thirtyfour children (92%) had the most common type of oesophageal atresia with proximal blind loop and distal tracheooesophageal fistula. Thirtyfour children were operated on, including one in Denmark. Nine children died within 60 days after surgery. The most common cause of death was lung inflammation (n=7, 78%). The survival after surgery in Iceland was 73% in the study period. Other congenital defects were common in this patient group with congenital heart defects as the most common ones (n=12, 32%). The incidence decresead in the study period from 1/3737 in the first ten years to 1/10639 in the last decade, this did not reach statistical signifiquance. Conclusion: It is interesting to see this decrease in incidence in the study period and this is the lowest incidence known to us. The survival has improved from previous study but is however still lower compared to our neighbouring countries. Other congenital anomalies are common in this patient group