Eagle syndrome includes elicitation of pain on swallowing, turning the head, or extending the tongue. The syndrome is thought to be caused by irritation of the glossopharyngeal nerve, most commonly caused by its impingement against an elongated styloid process. We present a rare case of a granular cell tumor presenting as Eagle syndrome. Granular cell tumors orignate from Schwann cells and are most common in the subcutaneous tissue of the head, neck, and oral cavity, especially the tongue. A granular cell tumor is typically benign and solitary, rarely malignant. The differential diagnosis, diagnostic algorithm, and treatment are presented.