Small cell neuroendocrine carcinoma(SNEC)is an extremely aggressive tumor and mainly occurs in the lung.Primary extra-pulmonary SNEC is rare.To date,only 11 primary SNECs occurring in the oral cavity have been reported in the English literature.We describe a case of primary SNEC of the right posterior tongue in a 46-year-old man.The patient had stage IVA disease and received adjuvant chemotherapy,followed by radical surgery and radiotherapy.He remained tumor-free for 20 mo before death due to gastrointestinal metastasis.The relevant literature on the 11 previously reported patients was reviewed,and the clinical features,histopathological characteristics,differential diagnosis and therapeutic strategies of this rare tumor were analyzed.