Small cell neuroendocrine carcinoma (SCNC) is an undifferentiated neoplasm that occurs primarily in the lungs. However, although extremely rare, it can also manifest in a variety of extrapulmonary sites, some of the most common being: prostate, urinary bladder, stomach, esophagus, salivary glands, larynx, gallbladder, skin and cervix. It is believed that these tumors develop from totipotent stem cells, which are present in all tissues. Furthermore, it can manifest as a metastatic disease from an unidentified primary site, with the head and neck region being the 4th most common site of extrapulmonary origin (16%)1. Extrapulmonary SCNC is histopathologically similar to those of the pulmonary site, and also has an aggressive biological behavior, marked by local recurrences and the early occurrence of distant metastases (especially to regional lymph nodes, bones and liver), with a 5-year survival rate of around 15%.1 In general, the initial symptoms are due to locoregional disease, but are indistinguishable from other tumors at the same site; symptoms can also be manifested by paraneoplastic syndromes and signs of distant metastasis. The approach varies according to the site of localization, presence of metastases and extent, however, treatment is generally performed surgically and/or through chemotherapy (usually containing etopside and platinum compounds) and radiotherapy, and aggressive locoregional therapy can be used in cases of localized disease, however, recurrences are common and adjuvant chemotherapy is