BACKGROUND Esophageal leiomyoma is a rare condition, with an estimated incidence rate of 0.4% of all esophageal neoplasms. These tumors are typically small, rarely more than 5 cm. The treatment depends on symptoms and the size and location of the tumor, with enucleation as the standard treatment of esophageal leiomyomas. Esophagectomy is performed only in very few cases, such as when the tumor is too large, there are multiple leiomyomas, there is a horseshoe shape or circumference, or the tumor is inextricably adhering to the esophageal mucosa. In such complex cases, it is often difficult to perform enucleation. However, with the risks of esophagectomy and intra-thoracic anastomosis, namely reflux, stenosis, leakage, abscess, and infection, attempting to perform enucleation for these cases should still be considered. CASE REPORT We reported a case of a large, multi-lobed, circumferential esophageal thoracoabdominal leiomyoma with successfully performed enucleation and esophageal preservation. A Dor fundoplication and Witzel jejunostomy tube were also performed. Follow-up 3 months postoperatively showed no appearance of reflux or dysphagia. The postoperative esophagogram visualized no obstruction or leakage. Histopathological results gave us concrete evidence of a leiomyoma: elongated cells with eosinophilic cytoplasm and rhomboid nuclei with uniform size. CONCLUSIONS The thoraco-laparoscopic enucleation approach is the method that should be considered first in the treatment of large, multi-lobed, circumferential esophageal leiomyomas, before contemplating esophagectomy and reconstruction.