Vagal body tumors (VBTs) are rare tumors of the paraganglion cells of the vagus nerve, usually occurring near the ganglion nodosum. They can be familial, multicentric, malignant, and sometimes hormonally active. The most accurate diagnosis is made by angiography. Primary treatment is surgical excision. Radiation therapy may play a role in preoperative preparation or in palliation. The prognosis is good when the tumor is found early and completely resected. Lifelong follow-up of patients and their family members is recommended.