Granular cell tumors are unusual neoplasms that were first described in 1926 by Abrikossoff.1 About 50% of granular cell tumors occur in the head and neck, and of these, more than half occur on the tongue,2-4 where they have been reported on the tip, dorsum, lateral border, base, and ventral surface.2 Other sites in the head and neck area include the buccal mucosa, lips, palate, larynx, trachea, orbit, esophagus, and cranial nerves.2,3 Most granular cell tumors are benign. Only about 2% exhibit malignant features.3-5 Metastatic granular cell tumors must be distinguished from local recurrences and benign multifocal tumors.5