Purpose <p>The primary objective of this study was to present a rare case of localized laryngeal amyloidosis in a pediatric patient, a condition with only 18 documented cases worldwide in individuals under eighteen years of age. Furthermore, it compares the diagnostic approach and management of this case with those outlined in the limited existing literature.</p> Methods <p>A 10-year-old male patient presented with progressively worsening dysphonia. Clinical evaluation included nasofibroscopy, contrast-enhanced CT imaging, and histopathological examination of resected tissue. The histological diagnosis was confirmed using Congo red staining. Additional tests were conducted to rule out systemic amyloidosis, including serum biochemistry and echocardiogram. The patient was followed for three years postoperatively.</p> Results <p>The patient was diagnosed with localized laryngeal amyloidosis, confirmed through histopathological analysis. Surgical management included a partial excision using cold dissection, followed by complete macroscopic resection with CO2 laser. Postoperative recovery showed minimal dysphonia with no dyspnea. Follow-up evaluations, including nasofibroscopy, revealed no significant recurrence of disease after three years, with only a small right posterior leukoplakia, interpreted as an impact-related lesion.</p> Conclusion <p>Localized laryngeal amyloidosis is an rare condition in pediatric patients. Early diagnosis is critical, and surgical management via transoral endoscopic resection is effective in improving symptoms. Regular follow-up is necessary due to the high likelihood of recurrence, especially within the first five years.</p>