Congenital oesophageal stenosis due to tracheobronchial remnants is a very rare condition characterized by the presence of tracheobronchial tissue in the oesophageal wall. The most common symptoms are dysphagia, regurgitation and hypersalivation. These usually appear in early infancy when solid food is introduced into the diet. Clinical diagnosis is difficult. Conventional radiology shows a dilated oesophagus without peristalsis, incomplete expansion of the gastro-oesophageal transition zone and delayed oesophageal emptying. Lesions suggesting oesophagitis may be present at endoscopy but pH-metry may be normal. The combination of stenosis with or without oesophagitis with normal pH-metry suggests that the patient's symptoms may be due to factors other than intraluminal and that further investigation is needed. The treatment of this condition requires surgical resection of the stenosis with little morbidity or mortality. The presence of tracheobronchial remnants is confirmed upon histopathological examination of the resected bowel segment.