This is the first report of a thoracoscopic esophagectomy for a giant leiomyoma of the upper esophagus aided by a robotic system. A 37-year-old man presented with progressive dysphagia and nocturnal aspiration. Endoscopic ultrasound and CT scan of the chest revealed an upper esophageal tumor of 9 x 4 cm arising from the muscularis mucosae. A fine needle aspiration showed clustering of mesenchymal cells, confirming the diagnosis of a stromal cell tumor. A mesenchymal malignancy was suspected because the tumor was located in the upper esophagus and was arising from the muscularis mucosae, both uncommon for a leiomyoma. Moreover, tumor size, an indicator of potential malignancy if >3 cm, was 9 cm. Therefore, an esophagectomy was performed thoracoscopically with the formation of a gastric conduit via laparotomy and a hand-sewn end-to-side cervical anastomosis. The thoracoscopic phase was performed with support of the da Vincitrade mark robotic system, which allowed for an excellent 3-dimensional view and a precise dissection of the esophagus along the vital mediastinal structures. The duration of the thoracoscopic part was 115 min and that of the total procedure was 270 min. Blood loss during the thoracoscopic phase was 50 mL; total blood loss was 200 mL. The patient was ventilated for 1 day; his total intensive care stay was 2 days. He left the hospital in good condition on the 11th postoperative day. Histopathological examination combined with immunohistochemistry revealed a leiomyoma of 9.0 x 5.0 x 2.5 cm. After 3 years of follow-up, the patient is in good health.