The purpose of this paper is to present and discuss a new case of malignant vagal paraganglioma. A 38-year-old woman was referred for the assessment of a bilateral firm cervical mass. CT scans revealed a left cervical ovoid mass; contralaterally, a mass with a similar enhancement could be observed too. The tumor was surgically removed via a transcervical surgical approach, together with levels I to III lymph nodes. Definitive histopathological examination proved the diagnosis of malignant vagal paraganglioma with contralateral cervical lymph node metastasis. One year after surgery, a PET-CT and a scintigraphy revealed the presence of a cervical recurrence and hepatic metastasis. Three months after a second surgical intervention, the patient died. Malignant vagal paraganglioma is a rare and challenging lesion that has to be carefully assessed to plan an appropriate treatment. A strict follow-up is fundamental for an early diagnosis of eventual recurrences.