The double bubble sign is a classic radiographic manifestation of duodenal obstruction, the cause of which could be intrinsic (such as duodenal atresia, duodenal stenosis or duodenal web) or extrinsic (such as annular pancreas or rotational anomalies).

Many infants with duodenal atresia also have Down syndrome. Duodenal atresia is often associated with other birth defects.