Summary:Paragangliomas are tumors that originate from the paraganglia tissue, which belongs to the extraadrenal chromaffin tissue. The glomus jugulare tumor is the most common benign tumor of the middle ear and the second most common tumor of the temporal bone. Hearing loss and pulsatile tinnitus are the most common symptoms of these tumors. The intravagale paraganglioma is a rare tumor of the head and neck, accounting for only 5% of the paragangliomas that occur in that area. A painless mass in the high neck with extension into the parapharyngeal space is the most common symptom of this tumor, which also has a striking tendency to be multicentric. Care must be taken to distinguish between glomus jugulare and carotid body or intravagale tumors. Likewise, the diagnosis of pheochromocytoma is crucial to avoid severe disarrangement in the perioperative period. Location in the jugular foramen with extension into the middle ear, neck, and intracranial space are pathognomonic of glomus jugulare tumors, but because occasionally meningiomas or neurinomas mimic these glomus tumors, an angiogram is necessary for obtaining an accurate diagnosis. Superselective embolization with absorbable material is an adjuvant therapy to surgery for glomus jugulare tumors, but meticulous care must be used to avoid the passage of embolic material into the arterial anastomoses of the vertebral artery with ascending pharyngeal artery and occipital artery. Despite numerous descriptions given in the literature concerning the efficacy of radiotherapy for controlling the growth of paragangliomas, the histological findings of these irradiated specimens have shown that the chief cells are not effectively affected by radiotherapy. Hence, the only curative treatment for paragangliomas is total resection. With current technique and management, this can be achieved without incidence of morbidity or mortality.