We report a case of small cell carcinoma of the esophagus(SCEC)treated by chemotherapy with 5-fluorouracil(5- FU)plus cisplatin(CDDP)and operation. A 60-year-old man was seen at the hospital because of a sensation of food sticking in his throat at meals. Gastrointestinal endoscopy revealed a type 2 tumor in the middle portion of the esophagus, which was pathologically diagnosed as small cell carcinoma. With a diagnosis of SCEC, preoperative chemotherapy with 5-FU plus CDDP was given. Primary lesion showed complete response. After chemotherapy, an operation was performed. The tumor had histopathologically disappeared, and the patient has been free of any signs of recurrence, as of 6 years 2 months after the operation. Primary small cell carcinoma of the esophagus is a rare disease with early systemic metastasis, and the prognosis remains poor. In a review of the literature, all patients with SCEC who had survived for 2 years or more had undergone combined therapies including operation, chemotherapy and radiotherapy. This study deals with a long-term surviving patient without any signs of recurrence after excision of the foci followed by chemotherapy.