Achalasia is a rare disease characterized by impaired lower esophageal sphincter relaxation loss and of peristalsis in the esophageal body. Endoscopic balloon dilation and laparoscopic surgical myotomy have been established as initial treatment modalities. Indications and outcomes of esophagectomy in the management of end-stage achalasia are less defined. A literature search was conducted to identify all reports on esophagectomy for end-stage achalasia between 1987 and 2017. MEDLINE, Embase, and Cochrane databases were consulted matching the terms "achalasia," "end-stage achalasia," "esophagectomy," and "esophageal resection." Seventeen articles met the inclusion criteria and 1422 patients were included in this narrative review. Most of the patients had previous multiple endoscopic and/or surgical treatments. Esophagectomy was performed through a transthoracic (74%) or a transhiatal (26%) approach. A thoracoscopic approach was used in a minority of patients and seemed to be safe and effective. In 95 per cent of patients, the stomach was used as an esophageal substitute. The mean postoperative morbidity rate was 27.1 per cent and the mortality rate 2.1 per cent. Symptom resolution was reported in 75 to 100 per cent of patients over a mean follow-up of 43 months. Only five series including 195 patients assessed the long-term follow-up (>5 years) after reconstruction with gastric or colon conduits, and the results seem similar. Esophagectomy for end-stage achalasia is safe and effective in tertiary referral centers. A thoracoscopic approach is a feasible and safe alternative to thoracotomy and may replace the transhiatal route in the future.