Small cell neuroendocrine carcinomas of tonsil are extremely rare cancers and only a few cases have been reported till date. They carry a poor prognosis. Paraneoplastic syndromes like SIADH, Cushing’s syndrome and Eaton-Lambert myasthenic syndrome can be found associated with these carcinomas. The tumor can metastasize to liver, lungs, bone, brain and skin. A 70-year-old male presented with a 2-month history of pain in throat which was moderate to severe in intensity and intermittent. Local examination of oropharynx revealed a 5×5 cm ulcero-proliferative growth over the right tonsil extending to right side of base of tongue, vallecula, and right retromolar trigone. Multiple matted lymph nodes were palpable in the right cervical region. Histopathological examination of tissue sample from the growth over the right tonsil revealed small cell neuroendocrine carcinoma. On immunohistochemistry, chromogranin and CD56 were positive. Patient received 2-courses of neoadjuvant chemotherapy with cisplatin and etoposide. Then the patient was planned for external beam radiation therapy with dose of 60 Gy in 30 fractions in 6 weeks. Small cell neuroendocrine carcinoma of tonsil are likely to be very aggressive with a tendency of developing early regional lymphatic and systemic metastases. More research and clinical trials shall be explored to obtain a standard treatment strategy for these patients.