The most common major birth defects affecting the oesophagus and trachea are oesophageal atresia (OA) and tracheo-oesophageal fistula (TOF). Epidemiological information on OA and/or TOF in the USA has been derived from only a few populations. Sixty-one cases of OA or TOF were identified through a population-based birth defects registry in Hawaii during 1986-2000. The relationship between various clinical and demographic factors and OA or TOF were examined. The rate of OA or TOF was 2.2 per 10,000 live births. The cases were 67.2% OA with TOF, 21.3% TOF alone, and 11.5% OA alone. Risk of OA or TOF was significantly higher among multiple births and live births with low birth weight and gestational age. The risk of OA or TOF was significantly higher for whites. The rate of OA or TOF was consistent with that reported elsewhere. The risk of the defects differed significantly between the major racial/ethnic groups in Hawaii. The impact of many factors on OA or TOF in Hawaii was similar to that reported elsewhere.