We evaluated a series of 5 patients-3 men and 2 women, aged 39 to 70 years (mean: 54.4)-with a granular cell tumor (GCT) of the head and neck in an effort to better define the clinical presentation, imaging characteristics, and surgical management of this type of tumor. In all cases, the diagnosis was established by pathologic analysis. There were 2 cases of laryngeal GCT and 1 case each of GCT arising in the nostril, hypopharynx, and the tongue base. The clinical findings were variable, depending on the location and extent of each lesion. Four of these patients underwent endoscopic examination, and in 2 cases computed tomography was performed. Treatment included wide surgical excision of the lesion in all cases. Otolaryngologists should be familiar with this unusual tumor. Although an accurate preoperative diagnosis is extremely difficult to make, appropriate therapeutic intervention is associated with a cure rate that is quite high.