Granular cell tumors of the larynx are relatively uncommon, always benign, and most commonly located in the posterior portion of the larynx. They are easily identified and should be differentiated from other lesions. A possible problem in the differential diagnosis is the presence of pseudoepitheliomatous hyperplasia, which overlies the granular cell tumor and which may mimic squamous cell carcinoma. Careful histopathologic differentiation is important because the laryngeal granular cell lesion should be managed conservatively, with transoral local excision usually being adequate. The histogenesis of these lesions remains in doubt, with a neural or epithelial derivation being the most likely possibility.