Congenital choanal atresia and stenosis (CA/S) are the most common craniofacial abnormalities of the nose. However, little is known about the epidemiology of these conditions. The present study was undertaken to expand our understanding of the epidemiology of CA/S by analyzing data from the Texas Birth Defect Registry (TBDR) for the period 1999–2004. Descriptive analyses and estimates of birth prevalence and crude prevalence ratios were used to characterize CA/S cases in Texas during the study period. Among the 2,209,758 live births in 1999–2004 in Texas, there were 202 cases of CA/S for an overall prevalence of 0.91 per 10,000 live births. The birth prevalence of isolated, multiple, and associated CA/S was 0.42, 0.21, and 0.28 per 10,000, respectively. The birth prevalence of isolated CA/S was similar in males and females, but was higher in the offspring of non-Hispanic white women (0.52/10,000), as compared to the offspring of non-Hispanic black (0.41/10,000) and Hispanic (0.37/10,000) women. There was also evidence of a significant association between birth year and isolated CA/S (P for trend = 0.0003), with lower risks observed in more recent years. No significant associations were observed between isolated CA/S and maternal age, education, residence along the Texas–Mexico border, plurality of the pregnancy or trimester in which prenatal care was initiated. These findings add to our limited understanding of the epidemiology of CA/S. © 2011 Wiley-Liss, Inc.