© 2015 The Authors. Journal of Paediatrics and Child Health © 2015 Paediatrics and Child Health Division (Royal Australasian College of Physicians). Aim A recent international study reported a higher prevalence of oesophageal atresia with or without tracheo-oesophageal fistula (OA±TOF) in Western Australia (WA). The aim of this study was to examine the prevalence and trends of OA and/or TOF in WA, determine the proportion of cases with associated anomalies and explore the impact of time of diagnosis. Methods The study population comprised all infants born in WA, 1980-2009, and registered with OA and/or TOF on the WA Register of Developmental Anomalies (WARDA). Results OA±TOF and TOF alone affect, on average, one in every 2927 births in WA, with a total prevalence of 3.00 and 0.42 per 10 000 births, respectively. The prevalence of OA±TOF increased by 2.0% per annum, with only cases with associated anomalies (64% of cases) demonstrating an increase. TOF rates were stable. Among OA±TOF infants, the proportion of live births, stillbirths and elective terminations of pregnancy for fetal anomaly (TOPFA) was 79%, 6% and 15%, respectively, whereas the majority (94%) of TOF only cases were live births. In 2000-2009, there was 30% fall in OA±TOF live births with 61 (58%) cases diagnosed in first week of life, 10 (9%) prenatally and 34 (32%) at post-mortem only. Conclusions A higher prevalence of OA±TOF in WA was observed with increase over time attributable to increase with associated anomalies. Consistent reporting, availability of prenatal diagnosis and ascertainment of cases following TOPFA or post-mortem examinations can significantly affect prevalence of OA and/or TOF.