Six-year follow-up of a case of small cell carcinoma of the esophagus showing a complete response is reported. The small cell pattern appeared intermingled with areas of invasive squamous cell carcinoma and carcinoma in situ. A neoadjuvant polychemotherapy regimen was used, followed by radiation therapy and adjuvant chemotherapy. This therapeutic combination resulted in complete response of the disease, as confirmed by computed tomography, endoscopy and biopsy. The patient is currently still alive, 6 years after the diagnosis, without any sign of relapse. A review of the literature reveals that this patient has survived longer than any others with this disease after treatment by chemoradiotherapy. The authors suggest that this therapy should be considered for patients with limited small cell carcinoma of the esophagus.