Introduction:
Paraganglioma are infrequent neuroendocrine tumors that are most commonly found in the carotid body, ganglia of the vagus, jugular and tympanic nerve. Very rarely they can involve other cranial nerves outside the cranial cavity, we present one such case of hypoglossal nerve paraganglioma in neck.
 
Case Report:
A 48 years old male presented with 1-month history of right sided stroke and aphasia. Ultrasonography of neck revealed a highly vascular mass on the right side of the neck. CT angiogram confirmed a highly vascular mass arising above the carotid bifurcation. With the working diagnosis of Glomus tumor, he underwent right sided neck exploration, however, intra-operatively tumor was found to be arising from the hypoglossal nerve instead. Surgery was abandoned on basis of the available literature, with only 6 reported cases in the past 54 years. Patient had no immediate post op complications and was sent for cyber knife treatment. After completion of 5 cycles of cyber knife there was a total of 45% reduction in the size of the paraganglioma with the resolution of the patient’s symptoms after a follow up of 6 months.
 
Conclusion:
Hypoglossal nerve paraganglioma is an uncommon tumor of the neck and can be misdiagnosed with the other tumors in this region especially chemodectoma and glomus tumor. The diagnostic criteria and appropriate treatment modalities have not been established due to the rare presentation hence hypoglossal paraganliomas should be kept in mind when Highly vascular neck mass is encountered.