Middle and lower esophagectomy was performed in a 68-year-old man in 1997 for the local recurrence of esophageal submucosal tumor after enucleation of the tumor. We subsequently performed partial hepatectomy for multiple liver metastases in 2001. The tumor was first diagnosed as GIST in the histological examinations of the liver metastases. However, 12 months after the hepatectomy, further recurrent lesions were found in multiple lymph nodes, in the remnant liver, and in the pancreas, as well as in the subcutaneous tissue. The lesions were initially considered unresectable, and we thus started an internal use of Glivec (400 mg/day) since July 2004. Within 2 months,all detected recurrent lesions changed into liquid forms,and the size of the subcutaneous tumor was also remarkably reduced. Accumulation of FDG was not seen on FDG-PET examination, suggesting the complete response of the tumor to Glivec. We still continue internal use of Glivec, and new recurrent lesions have not been detected up to now. Furthermore, the sizes of the liquid-regenerated lesions are gradually reducing.