Gastrointestinal neuroendocrine neoplasms are extremely rare. EsophagoGastric Junction (EGJ) is exceptionally struck by these neoplasms. The prognosis of these tumors is poor owing to their aggressive malignancy. A 77-year-old man was admitted with epigastric pain and dysphagia due to an ulcerative lesion of the EGJ, initially diagnosed by endoscopic biopsy as a moderately differentiated adenocarcinoma. Computed tomography scan revealed full thickness invasion of the EGJ and two abnormal lymph nodes along the lesser curvature of the stomach (cT2 N1 M0). An esophagectomy and partial gastrectomy with mediastinal and lesser curvature lymphadenectomy was performed. A cervical esophagogastric anastomosis completed the surgical procedure. The patient was discharge on the 11th postoperative day. Histology revealed a high grade neuroendocrine carcinoma combined with an adenocarcinoma of the EGJ (pT2 pN1 G3). The rarity of these tumors and the particularity of their heterogeneous cellular origin determine a diagnostic and therapeutic challenge not yet completely addressed.