Small cell neuroendocrine carcinoma (SNEC) is an uncommon tumor. This tumor usually occurs in the lungs, the extra-pulmonary form accounts for only about 4% of all cases. Primary SNEC of the paranasal sinuses is extremely rare; only about 76 cases have been reported in literature. Unfortunately due to the rarity of this neoplasm, there are no specific recommendations pertaining to the management, treatment options are generally extrapolated from similar tumors of pulmonary origin. While Surgery was used in the past, upfront chemoradiation now seems to be evolving as the treatment of choice. We report a case of sinonasal SNEC who had undergone definitive concurrent chemoradiation and is currently disease-free for close to 2 years. The clinical presentation, imaging studies, histopathological diagnosis with immunohistochemistry correlation, management protocols, and a brief review of literature of this rare tumor is discussed.Keywords: Management, paranasal sinus, prognosis, small cell neuroendocrine carcinoma
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