Neuroendocrine tumors (NETs) arise from neuroendocrine cells and are mostly observed in the gastrointestinal tract, pancreas, and lungs. NETs in the oral and maxillofacial region are extremely rare. We report a case of a 67-year-old man with an NET in the mandible. The patient did not show any symptoms except for remarkable jugular swelling. The lesion appeared as a radiolucent honeycomb abnormality with bone destruction on panoramic radiography. The histopathologic diagnosis following a biopsy was NET. Contrast-enhanced computed tomography (CT), 18Ffluorodeoxyglucose positron emission computed tomography (18F-FDG PET/CT), showed tumor mass of the soft parts of the right mandible with bone lysis without lymph node, visceral or bone hypermetabolism . He had three chemotherapy cures with good clinical and radiological response followed by concomitant radio chemotherapy 60Gy.The evolution was marked by a febrile grade IV aplasia leading to the death of the patient. Neuroendocrine carcinomas of mandible are unusual. Their prognosis is overall bleak. The morphological characteristics, the clinical aspects and the therapeutic management of these tumors are comparable to the neuroendocrine tumors of the lung.

Keywords : Neuroendocrine Small Cell Carcinoma, Mandible, Radiotherapy, Chemotherapy, Surgery.