OBJECTIVE: To report a case with bilateral jugulotympanic paraganglioma. CASE REPORT: An 83-year-old woman presented with complaints of pulsatile tinnitus for the past 10 years. The tinnitus was initially present in the left ear (LE) and then moved to the right ear (RE) and was associated with hearing loss, mostly in her LE. Otoscopy of the RE revealed a translucent tympanic membrane and a retrotympanic pulsatile purple-red mass. The red pulsatile tumor in the LE obliterated the LE canal (LEC). Magnetic resonance imaging (MRI) revealed a mass with hypercaptation located bilaterally in the jugulotympanic area, more extensive on the left, projecting itself on the posterior cranial fossa and the cerebellopontine angle cistern. Both lesions were heterogenic with hyperintensity at T2 and hypointensity at T1 and contrast enhancement. After the initial evaluation, surgical treatment, which is a definitive treatment, was not suggested due to the lesion extent and the poor clinical condition of the patient. The patient is currently being assessed for palliative radiotherapy. CONCLUSION: Glomus tumors or paragangliomas originate on glomus bodies, which are structures formed by the nonchromaffin part of the extra-adrenal neuroendocrine system, located in the jugular gulf region and the middle ear promontory. These are the most frequent benign neoplasms that occur in this region but are rarely bilateral. The main symptoms are hearing loss and pulsatile tinnitus. These symptoms should never be neglected and paragangliomas must be suspected and investigated in all cases, although a delay in diagnosis makes it harder or impossible to proceed with surgical treatment.