Esophageal gastrointestinal stromal tumors (GISTs) are very rare. We herein report our experience of three cases of esophageal GISTs. Two women and one man were treated by radical surgery either by thoracoscopic or by thoracotomy and thereafter followed up. Both histopathological and immunohistochemical analyses of tissue specimens showed positive findings for c-kit, which confirmed the diagnosis of GISTs. The malignancy of GISTs remains a clinical problem, and various indicators for the risk for recurrence or metastasis have been reported, such as tumor size, mitotic index, and mutation of the c-kit gene. As the tumor size was markedly large in two cases whereas the other had a high mitotic level, they are thought to have malignant potential. Because all three patients have demonstrated a disease-free status for at least 25 months, surgical therapy seems to be sufficient for such patients. However, further long-term follow-up may be necessary. © Japan Esophageal Society and Springer-Verlag 2005.