We experienced a case of esophageal leiomyoma combined with achalasia that is very rare. Patient had suffered from severe dysphagia and postprandial vomiting and diagnosis was accomplished by esophagography, esophagoscopy, chest CT, and esophageal motility test. The operative treatment was done through left lateral thoracotomy by enucleation of the submucosal tumor and esophagomyotomy. By histopathological findings, the diagnosis of leiomyoma was confirmed and LES biopsy revealed absence of the ganglion cells of myenteric and Auerbach`s plexus. Symptoms of the patient were completely relieved and postoperative course was uneventful.