INTRODUCTION: Neuroendocrine tumours of the larynx are extremely rare, though they make up only 1% of tumours in this region with the most common site being the supraglottis. On exhaustive research on PubMed, there are only a few prior cases of neuroendocrine carcinoma of subglottis published in the literature. CASE REPORT: A 56-year-old male presented to our center with complaints of hoarseness and dyspnoea for 1 month which was insidious in onset and gradually progressive. The patient was evaluated and diagnosed with as subglottic mass. He underwent tracheostomy followed by Micro laryngoscopy and Biopsy under GA. Post-op HPE report s/o Neuroendocrine carcinoma- small cell type. On evaluation no second primary or distant metastatic site was identified. CONCLUSION: Small cell neuroendocrine carcinoma of the subglottic larynx is an exceptionally rare and aggressive malignancy. Its presentation can be atypical, such as with symptoms of stridor, necessitating a high index of clinical suspicion. Early diagnosis and prompt, aggressive management are critical for improving outcomes. Despite the limited prognosis associated with this type of cancer, the patient in this case has shown a favourable response to chemoradiotherapy. Continued monitoring is essential due to the high risk of recurrence and metastasis associated with this malignancy.