Paragangliomas are rare in the larynx. When they do occur there, the most common subsite is the supraglottic compartment. Unlike other neuroendocrine tumors of the larynx, laryngeal paragangliomas are three times as common in women as in men. Although a preoperative biopsy is often performed to establish the diagnosis, this procedure carries a considerable risk of bleeding, which may necessitate a tracheotomy to secure the airway. Immunohistochemical staining is useful in the differential diagnosis to distinguish a paraganglioma from other neuroendocrine tumors. Computed tomography and/or magnetic resonance imaging with preoperative angiography and possible embolization are important to obtain prior to treatment. Surgical excision is the treatment of choice.