Granular cell tumors are rare neoplasms that arise in different regions of the body. Their cell of origin is still debated among many pathologists, but recent authors consider them to be neurogenic. These tumors are believed to be benign; however, local recurrences after excision and multifocality arouse suspicions for malignancy. In the head and neck, granular cell tumors occur most frequently in the tongue. In the laryngotracheobronchial tree, most tumors reported have been in the larynx and the next most in the bronchi. Only six cases of true tracheal occurrences have been reported previously; two additional cases are reported in this paper. The literature of the cell of origin of these tumors is reviewed, the different theories are presented, and diagnosis, treatment, and follow-up are discussed.