Small cell carcinoma of the esophagus is reported to be both rare and aggressive, and is commonly associated with a short post-diagnosis survival period. We report a case of Stage IV small cell carcinoma of the esophagus involving a relatively long survival period of 2 years. The patient was a 70-year-old man with a chief complaint of epigastric discomfort. Upper gastrointestinal endoscopy by a local physician showed a protruding tumor at the mid-thoracic esophagus. Tumor biopsy revealed small cell carcinoma. Chest-abdominal computed tomography revealed multiple liver metastases, and swelling of a number of regional lymph nodes. The diagnosis was cT3N4M1, cStage IVb esophageal small cell carcinoma. According to the regimen used for lung small cell carcinoma, we administered CDDP/CPT-11. After administration of four courses, the primary lesion, liver metastases, and lymph node metastases significantly reduced, indicating a partial response. However, after completion of eight courses, liver metastasis and lymph node swelling recurred. The patient subsequently received other therapeutic regimens such as CBDCA/VP-16, AMR, NGT, and CDGP/5-FU. However, the tumor gradually progressed, and the patient died approximately 2 years after the first treatment.