Aims: Primary small cell neuroendocrine carcinoma (SCNC) of the larynx is an exceptionally rare and aggressive tumour, frequently associated with poor prognosis and advanced disease at presentation. We present a rare instance of this pathology describing its clinical presentation, diagnostic challenges, and therapeutic management. Additionally, we review 260 cases from the literature to assess survival outcomes and suggest optimal treatment strategies for this malignancy. Methodology: A 50-year-old male with a history of smoking presented with progressive dysphagia, dysphonia, and dyspnea. Diagnostic workup investigations included a computed tomography scan, positron emission tomography scan, endoscopic evaluation, biopsy, and immunohistochemistry. The patient was diagnosed with SCNC of the supraglottis, with cervical lymph node metastasis (T2N1M0). A multidisciplinary team recommended concurrent chemoradiotherapy. The patient underwent chemotherapy with cisplatin and etoposide, alongside radiotherapy to the larynx and bilateral neck. Results: Given the rarity of SCNC and the scarcity of randomised controlled trials, treatment approaches are generally adapted from protocols used for small-cell lung cancer. A multidisciplinary approach, primarily focused on combined chemoradiotherapy, has shown the best outcomes in terms of survival while preserving laryngeal function. Despite our patient remaining disease-free post-treatment, our literature review of 260 cases indicated a disease-specific survival rate of 33.1%, highlighting the aggressive nature of SCNC. Conclusion: SCNC should be considered a key differential diagnosis for submucosal laryngeal masses. This case highlights the importance of early diagnosis and treatment for this rare laryngeal pathology in an adult patient. Further high-quality, multicenter research is required to advance our understanding of the management of these tumors.