Three patients with fibromuscular oesophageal stenosis are reported. The onset was usually before 6 months of age; earlier than that of stenosis due to tracheobronchial remnants. The localization of the stenotic area varied from the distal to the mid-oesophagus. Extramucosal myotomy seemed to be the treatment of choice for this lesion, particularly in the mid-oesophagus involved diffusely, because of its good effectiveness, less invasiveness, and simplicity.