Small cell carcinoma is a rare, highly aggressive and locally invasive, poorly differentiated neuroendocrine carcinoma that mainly occurs in the lungs. Extrapulmonary small cell carcinoma of head and neck is uncommon. It is extremely rare to occur in the tonsil. These tumors are often seen in male smokers in their seventh decades. Definite diagnosis relies on pathologic examination. Treatment of these tumors is often difficult because of rapid growth and early metastases. Due to high incidence of distant metastasis at the time of diagnosis, combined treatment with systemic chemotherapy and locoregional radiation therapy is the treatment of choice. The prognosis of the tumor is generally considered as very poor. We presented 54-year-old man with small cell carcinoma of tonsil and reviewed the pertinent literatures.