In the head and neck mucosa, neuroendocrine carcinomas of the oral cavity is rare. Herein, we present the first report of a small cell neuroendocrine carcinoma in a 54-year-old man on the right lateral posterior tongue. It is important to remember that although neuroendocrine small cell carcinomas (SCCs) are most commonly seen in the lung, they rarely may arise in the extrapulmonary sites, including salivary glands, as well. As there is not any standard therapeutic regimen already existing, it is important to be aware of and to know how to deal with such rare cases.