Although leiomyoma is the most common esophageal mesenchymal neoplasm, it is a rare condition. Resection of the tumor is recommended in symptomatic patients, and observation is recommended in asymptomatic patients with small lesions. We discuss herein a patient admitted to our outpatient clinic for dyspepsia in whom a giant annular esophageal leiomyoma was diagnosed. Resection through thoracoabdominal approach is recommended instead of adjuvant treatment in similar sized tumors.