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Primary gastrointestinal small cell carcinoma is reported 0.1%–1.0% of malignant gastrointestinal tumors and predominantly in the esophagus. All reported cases are in the adult population. We present a 9-year-old boy with small cell neuroendocrine carcinoma of the esophagus with mediastinal lymph node involvement, whose chief complaint was progressive dysphagia. He survived for 22 months with chemoradiation but did not have resectional surgery. Although small cell neuroendocrine carcinoma of the esophagus is extremely rare in children, it should be considered in the differential diagnosis of any undifferentiated tumor of the esophagus in any age.