We present a case of small cell esophageal carcinoma (SCEC) treated with alternated chemotherapy (including cisplatin, etoposide, vincristine, cyclophosphamide and doxorubicin) and irradiation (36 Gy) followed by surgery. Despite a pathological complete response, the patient died of regional disease recurrence 29 months after the diagnosis. We reviewed the available literature on SCEC with regard to the incidence, clinical symptoms, radiological signs, diagnostic workup, therapeutic modalities and prognosis of this malignancy.