A 31 year-old female initially presented with clinical features consistent with achalasia, which were relieved by the Heller procedure. Several years later manometric tracings excluded the diagnosis of achalasia, and suggested a motility disorder of the esophageal body. A long esophageal myotomy was performed and was followed by a marked improvement in symptoms and a normalization of the manometric tracing. Multiple histologic sections from the surgical specimen revealed the absence of ganglion cells, a feature not previously described in motor disorders other than achalasia. These findings suggest that classification of esophageal motility disorders on the basis of manometric and histologic findings might not be possible and that surgical treatment should be directed towards the predominant symptoms.