A 59-year-old male visited our hospital with complaints of difficulty in swallowing. X-ray barium studies and upper gastrointestinal endoscopic examination revealed an irregular ulcerated lesion in the lower portion of the esophagus, which was diagnosed based on pathology tests as small cell carcinoma. A computed tomography scan showed para-aortic lymph node swelling and multiple liver metastases. Treatment with chemotherapy of CDDP and 5-FU showed clinical complete remission. However, the patient died of paraaortic lymph node metastasis, recurrence of the original lesion, multiple liver metastasis and brain metastasis 23 months after diagnosis. The prognosis of small cell carcinoma of the esophagus is quite unfavorable because of the highly aggressive biological behavior. However, if remission is achieved by chemotherapy as in this case, a better prognosis is possible.