A-13-urar-old with a history of Alport syndrome had been suffering from progressive dysphagia and postprandial vomiting for over 7 years. Exophagogram and manoemtric studies were consistent with achalasia. Barum study demonstrated marked esophageal dilatation and smooth tapered narrowing of the distal esophagus. However in spite of the medical treatment including the injection of the distal esophagus. However in spite of the medical treatment including the injection of botulinum toxin at the lesion site using an endoscope symptom did not improved and he suffered growth failure and malnutrition. Esophagectomy and esophagogastrostomy were performed to relieve the dysphagia. A firm circumferential intramural mass about 7$ imes$5$ imes$5 cm was found in the distal esophagus. The lumen of the esophagus was markedly dilated and esophageal wall was hypertrophied. Histologic examination of the neoplasm revealed a rather ill defined tumor tissue consisting of interlacing or whirling spindle cells without significant mitosis and esophageal leiomyo-matosis was confirmed. The patient was discharged uneventfully.