A 16-month-old boy presented with a story of stridor, solid dysphagia and a history of spectacular protrusion underneath his tongue which was mistaken by the parents for a snake's tongue! The radiological examinations showed a filling defect of the upper and middle third esophagus which compressed the cervical trachea. This was found to be an intraluminal tubular esophageal duplication. Treatment by cervicotomy and unusual histological facts are presented.