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The most common site involved in the head and neck region is the larynx and the oropharynx as a primary site has been infrequently reported. The patient presented with bilateral neck swelling and an ulcerated lesion was noted in the base of tongue (BOT). A biopsy revealed small cell carcinoma (SCC) and was confirmed with immunohistochemistry. The final diagnosis after workup was SCC of BOT stage IVc. He received induction chemotherapy to which the tumour responded partially, followed by curative intent chemoradiotherapy and adjuvant chemotherapy. The patient achieved a radiological complete response to treatment and almost all treatment-induced toxicities resolved. 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