Paragangliomas of the larynx are rare neuroendocrine tumors with difficulties in diagnosis and management. A review of the literature was carried out, using Medline and other available databases. Electronic links and related books were also included. The most reliable diagnostic imaging procedures are MRI, CT scan, and octreotide scintigraphy. Complete surgical excision with the maximal possible preservation of the laryngeal function is the treatment of choice. Many different surgical techniques have been reported but open surgical procedures seem to provide better results with lower recurrence rates, although endoscopic approaches and laser surgery have also been used with variable results. The use of long-acting depot octreotide has been used for the relief of symptoms and stabilization of the disease, especially in inoperable lesions with positive initial octreotide scintigraphy imaging. Detailed preoperative assessment and treatment planning in individual basis are essential in the management of these tumors.