Background: Jugulotympanic paragangliomas is a rare tumor that generally presents in the 5th or 6th decades of life with pulsatile tinnitus and

hearing loss. In this report, we present an unusual case of jugulotympanic paraganglioma presenting in 48 year old man with multiple cranial nerve

palsy and cerebellar ataxia.

Case Report: A 48 year old man presented with diplopia with worsening episodes of dizziness. He also complained of right sided tinnitus and

hearing loss along with difficulty in degglution, dysarthia, decreased sensation from right half of face and atropy of right sided tongue muscles.

Examination reveals red bulging tympanic membrane right sided along with multiple cranial nerve palsies. CT head suggestive of right sided

jugulotympanic paraganglioma which on the MRI shows multiple intracranial and extracranialexpansion with numerous vessels forming AV

malformations within the lesion, extending from CP angle down along jugular vein with epicenter in jugular foramen.

Conclusions:Jugulotympanic paragangliomas are rare tumors that usually manifest in middle age with tinnitus and hearing loss. However,

clinicians should be aware of unusual presentation of this tumor if presented with multiple cranial nerve palsy and cerebellar ataxia. All the above

described clinical features should trigger further investigations with a CT scan or MRI of the brain.