Introduction: Granular cell tumor is an unusual growth of probably neuroectodermal histogenesis, first reported by Abrikossoff in 1926 with the name of myoblastoma. About 1.200 cases were reported since then, 50% in the head and neck. Of these, 10% have a laryngeal localization. Objective: To describe a case of a patient with diagnosis of laryngeal granular cell tumor. Case Report: A 34 year-old female patient presented with a neck mass as a first sign. Examination with a rigid laryngoscope demonstrated an irregular red hypertrophy lesion in the posterior third of subglottic region. The tumor was treated successfully by total laryngectomy. Conclusion: A granular cell tumor may present as clinically innocuous lesions on the larynx and the diagnosis is usually made by histopathology. The differential diagnosis should include squamous cell carcinoma.