Diagnosis of oesophageal stenosis, especially in a child, should incline one to consider whether it is a congenital or secondary disorder. In this report the course of the disease in a girl with Down’s Syndrome in whom oesophageal stenosis was recognized at the age of 12 is described. A contraction of the oesophagus was detected through 25% of the length of the oesophagus. The probable causative congenital nature of stenosis and its relation with gastroesophageal reflux is also discussed.