Esophageal neuroendocrine carcinoma is relatively rare and the prognosis is poor owing to its aggressive malignancy. We experienced a case of neuroendocrine carcinoma of the esophagus treated with neoadjuvant chemotherapy of 5-fluorouracil plus cisplatin. A 71-year-old man was diagnosed with esophageal carcinoma(clinical T3N1M0, Stage III ). He received neoadjuvant chemotherapy comprising 5-fluorouracil plus cisplatin, followed by subtotal esophagectomy with dissection of 3 regional lymph nodes. Immunohistochemical findings were synaptophysin-positive, CD56-positive, Ki-67(labeling index) B90%. The final diagnosis was neuroendocrine carcinoma, categorized as CT-pT1a-MM, pN0, Stage 0. The pathological response to neoadjuvant chemotherapy was Grade 2. The patient is alive 8 months after esophagectomy without metastasis.