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Small cell neuroendocrine carcinoma (SCNEC) of the paranasal sinuses is extremely rare, with an unclear pathogenesis. The presence of neuroendocrine granules is suggestive of neuroendocrine differentiation. It was reported that this disease relates to the presence of accessory salivary glands, and some basic research has shown that it might originate from the multi-potent stem cells.There are no specific clinical symptoms but rhinal and ophthalmological symptoms are found in most cases.Diagnosis mainly depends on histopathological manifestations.Immunohistochemical results and features of the electron microscopic ultra.Structure.Pathologfcal differentiation from poorly differentiated squamous carcinom,melanoma,esthesioneuroblastoma and neuroglioma etc.is needed.No unified regimen has been employed in treating the disease.At present,combined theraPY has a manifest therapeutic effect.such as success with the 2003 French regimen.Tumor relapse iS common and prognosis is poor.A complete combined treatment plan will be helpful to improve the prognosis.