Recurrent tracheoesophageal fistula (TEF) is still difficult to diagnose and repair. In almost all cases, recurrence appears relatively soon after the primary surgery. We herein describe a case of recurrent TEF that appeared 10 years after the primary repair. At 2 years of age, the patient suffered from mental retardation due to encephalitis and developed a hiatus hernia with gastro-esophageal reflux. He underwent the repair of a hiatus hernia and fundoplication at 3 years of age. However, the hiatus hernia recurred 6 months after the operation. The patient suffered from recurrent pneumonia for 6 years after the appearance of the recurrent hiatus hernia. At 9 years of age, he was hospitalized frequently due to recurrent severe pneumonia. After admission at 9 years of age, an endoscopic study under general anesthesia was performed and revealed subglottic stenosis and a dilated esophagus with a recurrent hiatus hernia. Tracheotomy or laryngotracheal separation was first planned in order to improve his upper airway and facilitate the safer repair of the recurrent hiatus hernia. After laryngotracheal separation, the patient still suffered from severe pneumonia. In addition, a small volume of nutritional supplement was aspirated from the tracheostomy. Thus, recurrent TEF was suspected. Tests using dye under both esophagoscopy and bronchoscopy confirmed recurrent TEF. The fistula recurred in the cervical area because of the elevation of the esophagus due to the recurrent hiatus hernia. The fistula was surgically closed, with a sternothyroid muscle flap to prevent re-recurrence. At 4 months after this operation, the recurrent hiatus hernia was repaired. Thereafter, the patient’s respiratory symptoms showed a dramatic improvement. The patient is now doing well and free from further recurrences of TEF and hiatus hernia at 2 years after the final operation.