Small cell carcinoma of the esophagus is a relatively rare disease, and its prognosis is quite poor, because of its rapid progression. A 76-year-old female visited our hospital with the complaint of a poor appetite. Upper gastrointestinal endoscopic examination(GIS)revealed an ulcerated lesion in the lower portion of the thoracic esophagus, and we diagnosed small cell carcinoma by a biopsy specimen. A computer tomography scan revealed solitary liver metastasis, and lymph node swelling on the left side of the superior mesenteric artery. So, we started chemotherapy with VP-16 and CDDP, according to a regimen for small cell carcinoma of the lung. After 4 courses of chemotherapy, the primary lesion, liver metastasis, and lymph node swelling had disappeared, so we decided it was a complete response. 20 months later, follow-up GIS revealed low elevated lesion at the margins of an ulcerated scar, and diagnosed squamous cell carcinoma by the biopsy specimen. The patient received 60 Gy radiotherapy in total, and is still alive 6 years after diagnosis without any evidence of recurrence.