A 47-year-old patient presented with a history of dysphagia for solid food for almost 10 years and weight loss of more than 50 kg. Non-resecting surgical as well as endoscopic procedures (laparoscopic cardiomyotomy with secondary antireflux operations, balloon dilation, Botulinum-toxin injection) were without success. A barium esophagogram showed a confinement of the distal esophagus with a filiform passage of the contrast medium and undigested food in the prestenotic dilated esophageal corpus. Manometry displayed a hypertensive lower esophageal sphincter with a resting pressure of 43.8 mmHg - although completely relaxing. The tubular esophagus was aperistaltic with 100 % simultaneous and repetitive contractions. As all attempts of previous therapy had failed, a transhiatal esophagectomy with gastric pull-up and cervical esophagogastrostomy ensued. Neuropathological examination of the esophagus showed that degeneration of the myenteric plexus was not severely involved, whereas inflammatory and fibrotic changes were obvious. Esophageal resection provided the only chance of a long-term benefit for our patient with relief of dysphagia.