OBJECTIVE Granular cell tumor of the larynx is an uncommon, typically benign lesion that may be confused for a malignant neoplasm based on histopathology. This review examines cases of granular cell tumor of the larynx in adults to highlight key distinctions in diagnosis/management and demonstrate how misclassification may lead to unnecessary escalations in therapy. METHODS A systematic search of PubMed, Ovid, and EBSCO Search Hosts was completed in December 2021. The search yielded 501 articles with 87 full-text articles included in the review. Primary search terms included granular cell, tumor, larynx, and adult. Primary endpoints were patient presentation, primary management, pathological features, and disease course. RESULTS A systematic review of 87 articles identified 200 patients with granular cell tumors (GCTs) of the larynx. Of the 200 patients, 50.3% were males and 49.7% were females. Of these, 54.0% were reported as white patients, and 46.0% were reported as black patients. The most common presenting symptoms were dysphonia (85.9%) and stridor/dyspnea (14.1%). On examination, the lesions were most commonly polypoid/nodular and firm. Pseudoepitheliomatous hyperplasia (PEH) was identified in 33.5% of cases, and 2% of cases were malignant. GCTs were misdiagnosed as other malignant lesions in 11% of cases. In benign cases, 13.5% of patients underwent additional surgeries beyond simple excision/laryngofissure, including laryngectomy and neck dissection. Less than 2% of lesions reoccurred. CONCLUSIONS Granular cell tumors of the larynx are typically benign lesions that may be misdiagnosed with unnecessary escalation of treatment. However, most lesions resolve via primary surgical excision. Laryngoscope, 134:1523-1530, 2024.