Gastrointestinal stromal tumor is documented on every part of the gastrointestinal tract. It develops in the stomach and the small intestine most commonly, and also in the esophagus. A 44 year-old male patient was admitted due to dysphagia and weight loss. Chest CT showed about $15 imes11 imes11cm$ sized, well-defined, and lobulated soft tissue mass with central necrosis was noted in the posterior wall of lower esophagus throughout the lesser curvature of upper stomach. We performed the distal esophagectomy and total gastrectomy using thoracoabdominal incision. The tumor was positive at CD117 (c-kit) and CD 34, and was diagnosed as malignant GIST of the distal esophagus and upper stomach. The patient is on routine follow up at the out patient department for nineteen months up to now.