RATIONALE: Small cell neuroendocrine carcinoma (SCNEC) of the larynx is a rare and aggressive malignancy, notorious for its rapid progression and poor prognosis. Early diagnosis and effective management are crucial, particularly for high-risk populations such as long-term smokers. PATIENT CONCERNS: A young male smoker presented with progressive laryngeal symptoms, which prompted further investigation due to the severity and progression of his symptoms. DIAGNOSES: The patient was diagnosed with SCNEC of the larynx after presenting with symptoms. Diagnostic procedures confirmed the presence of a localized tumor without distant metastasis. INTERVENTIONS: In response to the diagnosis, the patient underwent a surgical intervention that achieved clear margins, chosen based on the tumor's localized nature at diagnosis. This approach emphasized comprehensive initial surgical management. OUTCOMES: The absence of immediate postoperative recurrence highlights the potential effectiveness of the surgical approach as a standalone treatment in selected cases of SCNEC. LESSONS: This case underscores the importance of considering SCNEC in the differential diagnosis when young smokers present with laryngeal symptoms. It highlights the need for proactive surgical strategies to manage this aggressive cancer effectively. Additionally, it emphasizes that despite the favorable initial outcome, the high risk of recurrence necessitates rigorous long-term follow-up and a structured surveillance strategy to enhance early detection and potentially improve long-term outcomes. This report offers significant insights into the rapid progression and nuanced management of SCNEC, providing valuable lessons for clinical practice.