Neuroendocrine tumours are rare and have been reported to arise in a number of structures within the head and neck. We present the case of a 55-year-old lady who presented a T1-N2-M0 neuroendocrine tumour of the tongue and right level 2A lymphadenopathy. The patient underwent a partial right-sided glossectomy and a modified radical neck dissection. Given the rarity of small cell neuroendocrine tumours of the tongue, there is some ambiguity with respect to classification. Treatment for neuroendocrine tumours is most effective with a multimodality approach and a poor response to chemotherapy is an important prognostic indicator. Radiotherapy, combined with chemotherapy, has shown the most promise with complete resolution of the primary tumour and metastatic disease. Due to the rarity of neuroendocrine tumours and the lack of favourable prognostic indicators, defining optimal treatment remains difficult. As a result, they continue to have a poor overall prognosis.