Abstract: Objective To analyze the clinical manifestation, diagnosis, treatment and prognosis of neuroendocrine carcinoma (NEC)in the oral cavity. Methods Two cases of primary NEC admitted to our hospital were reported, and related literatures were reviewed. Results Case No.1 was a 60-year-old women with primary neuroendocrine tumor of small cell type confirmed by histological and immunohistochemistry staining results. Case No.2 was a 74-year-old women with poorly differentiated primary NEC arising in the mandible. Both two cases had neck lymph node metastasis before diagnosis. Partial jaw resection and neck resection under general anesthesia were performed. Regrettable, the two patients died of NEC at 20 months and 38 months after surgery respectively. Conclusion NEC in the oral cavity is highly aggressive, often relapses locally and causes early distant metastasis with poor prognosis.