We present the third case described to date of large cell neuroendocrine carcinoma located at the esophagogastric junction (LCNEC). Esophageal neuroendocrine tumours account for 0.03-0.05% of all malignant esophageal tumours. Within oesophageal NETs, LCNEC accounts for 1% of esophageal NETs. This tumour type is characterised by elevated levels of certain markers: synaptophysin, chromogranin A and CD56. In fact, 100% of patients will have chromogranin or synaptophysin, or at least one of these three markers. In turn, 78% will have lymphovascular invasion and 26% will have perineural invasion. Only 11% of patients will have stage I-II, which implies an aggressive course and worse prognosis.