Congenital duodenal stenosis is one of the most common causes of neonatal obstruction, which is frequently associated with annular pancreas and Down syndrome. Ectopic pancreas is defined as an abnormally situated pancreatic mass that lacks contact with normal pancreas. Although the association between duodenal stenosis and annular pancreas is very common, the presence of an ectopic island of pancreas in such cases is very rare. We present a case of unusual association of duodenal stenosis, jejunal ectopic pancreas in a neonate with Down syndrome.