Duodenal obstruction is one of the most common congenital anomalies. Both intrinsic (atresia, web and stenosis) or extrinsic lesions (malrotation, peritoneal bands, intestinal duplication, anterior portal vein and annular pancreas) may be involved. It usually presents within the first week of life with vomiting. We report an unusual congenital duodenal obstruction in a 8-month-old female with atypical symptoms due to a combination of intrinsic and extrinsic lesions.