PurposeThis study aims to identify the prevalence of Congenital abdominal wall defects (AWD), hospital outcomes, and related congenital abnormalities in Taiwan by using the National Health Insurance Research Database (NHIRD). Materials and methodsFrom 1998 through 2013, all pediatric patients with AWD were collected via ICD-9-CM diagnostic code 756.7x (“congenital anomalies of abdominal wall”) or procedure codes (54.71, 54.72 for Gastroschisis repair (GS-repair); 54.63, 53.41, 53.49 for other abdominal wall repair (O-AWD)) recoded in NHIRD. We used public national birth data for calculating the prevalence. We used CDC/BPA coding rubrics to identify accompanying congenital abnormalities. ResultsA total 594 babies with AWD, including 179 patients in GS-repair group and 326 patients in O-AWD-repair group, are identified with 113.5 months median followed-up. The overall prevalence for AWD was 1.65 in 10,000 births and decreased over time. The prevalences of GS-repaired and O-AWD-repaired are 0.50 and 0.90 in 10,000 births. The 1-year-mortality rates of GS-repair and O-AWD-repair are 6.15% and 7.23%. 24.58% GS-repair patients and 30.06% O-AWD-repair patients have congenital abnormalities. 8.38% GS-repair patients and 6.44% O-AWD-repair patients received the following groin hernia-repair procedures. ConclusionOur results showed that the prevalence of AWD was low and decreased over time in Taiwan. Level of evidenceLevel II: Prognosis Study, Retrospective study.