Objective To study the diagnosis and treatment of neuroendocrine carcinoma of the nasal cavity and paranasal sinuses, and analyse the influencing factors of suvival and prognosis. Methods We retrospectively analyzed the diagnosis and treatment process of 14 patients with sinonasal neuroendocrine carcinoma (SNEC) admitted in The First Affiliated Hospital of Kunming Medical University from 2007 to 2011. All patients were followed up to learn the survival status of them.Results All patients were followed up for one year up to six years except 2 patients who gave up treatment. Five patients died and six survived with good tumor control in the followed up period. Two patients received only endoscopy surgery, and one of them died from lung metastasis in 21 months after operation, and the other one survived with good tumor control, the disease free survival (DFS) was 9 months . Eight patients were treated by endscopy surgery and /or chemo-radiotherapy, three cases died in following-up period, and five of them survivied with good tumor control, and the disease free survival was 20.25 months.Two patients with transcatheter arterial infusion chemotherapy survivied with good tumor control within the follow up period,and the DFS was 25.5 months.Five patients had moderately differentiated SNEC and DFS was 25.5 months. Seven patients had poorly differentiated SNEC with DFS 14.6 months. Six patients were T4N0M0, four patients were T3N0M0, two patients T2N0M0, and their DFS were 19 months, 12.8 months and 33 month, respectively. Conclusions Surgery with radiotherapy and/or chemotherapy is the current treatment method for sinonasal neuroendocrine carcinoma. Small cell neuroendocrine carcinoma with poor differiation displays highly aggressive and poor prognosis. Diagnosis and treatment in early stage is important for good prognosis.