Collet-Sicard syndrome is caused by various neoplastic and non-neoplastic lesions affecting the base of the skull with involvement of IX, X, XI and XII cranial nerves. Paraganglioma accounts for <1% of all the neoplasms in the head and neck region. They are traditionally considered as benign, slow growing, locally invasive, encapsulated and highly vascular tumors. We report a case of Collet-Sicard syndrome secondary to a large glomus jugulotympanicum in a 45-year-old woman who presented to the emergency department with complaints of recurrent episodes of a fresh bleeding from the left ear for the previous 5 days. She had pain and decreased hearing for the last 3 years and features of multiple cranial nerve palsies. A radiological diagnosis of glomus jugulotympanicum (paraganglioma) was made, which was confirmed by the biopsy tissue. At 6-month follow up, episodes of recurrent bleeding had stopped, but cranial nerve palsies persisted.