A diagnosis of diffuse esophageal spasm (DES) based on radiological and manometric studies was made in a 70-year-old man who presented with severe dysphagia, vomiting, and spontaneous chest pain. The manometric studies revealed a simultaneous onset of high amplitude contractions and a hypertensive lower esophageal sphincter (LES) that was well relaxed in response to deglutition, in contrast to the incomplete relaxation seen in achalasia. Because his dysphagia was so severe and did not respond to pneumatic dilatation, the patient was treated by a long esophageal myotomy with a full thickness incision through the LES and mucosa, adding a Thal-Hatafuku procedure. The patient made a good postoperative recovery and has since been eating normally without any further dysphagia or chest pain. Good manometric and radiological results have been obtained in this patient during 5 years of follow-up.