BackgroundFew studies of congenital anomalies provide prevalence estimates stratified by maternal race/ethnicity. We sought to determine whether the prevalence of a broad spectrum of anomalies varies among offspring of women from different race/ethnic groups.MethodsWe obtained information on cases with anomalies from the population‐based Texas Birth Defects Registry, and denominator data on livebirths among Texas residents during 1999–2018 from the Texas Center for Health Statistics. We estimated the prevalence ratio (PR) and 95% confidence interval (CI) of N = 145 anomalies among offspring of Hispanic and non‐Hispanic Black relative to non‐Hispanic White women using Poisson regression, adjusting for maternal age, education, body mass index, and previous livebirths. We performed a two‐stage analysis with a Bonferroni‐adjusted p < 1.7 × 10−4 in the initial screening phase to identify anomalies with statistically significant variation.ResultsThere were 7,698,768 livebirths and 1,187,385 anomalies diagnosed in 368,393 cases. The prevalence of any monitored congenital anomaly was similar among offspring of non‐Hispanic White (referent), non‐Hispanic Black (PR 0.98, CI 0.96–1.00), and Hispanic (PR 0.95, CI 0.93–0.96) women. We observed statistically significant racial/ethnic variation for 42 anomalies. Marked differences were observed when comparing offspring of non‐Hispanic Black to non‐Hispanic White women with respect to polydactyly (PR 4.38, CI 4.07–4.72), pyloric stenosis (PR 0.34, CI 0.29–0.40), and aortic valve atresia/stenosis (PR 0.51, CI 0.36–0.72).ConclusionsBirth prevalence of many major congenital anomalies varies by maternal race and ethnicity. While the reasons for these differences are likely multifactorial, a thorough understanding of racial and ethnic disparities is useful to stimulate etiologic research.