Granular cell tumor is a rare, usually benign neoplasm that can occur in various parts of the body. Half of all granular cell tumors occur in the head and neck, with 33% of these occurring in the tongue. The larynx is relatively an uncommon location, accounting for approximately 7% to 10% of all reported cases. Typically, the most common presenting symptom is hoarseness, with some patients also presenting stridor, hemoptysis, dysphagia, and otalgia. But the tumor may be asymptomatic and discovered only incidentally during a routine examination. The diagnosis is usually made on the histopathological findings, characterized by abundant eosinophilic cytoplasm filled with granules of varying sizes. Complete excision with an attempt to maintain normal structures generally results in cure. We experienced a 16-year-old man with laryngeal granular cell tumor, which involves the right true vocal fold. He was successfully treated with local excision under laryngomicroscopic fine dissection. So we present this case with a review of literature.