To the best of our knowledge, only 2 cases of a simultaneous contralateral vestibular schwannoma (VS) and middle ear paraganglioma (MEP) have previously been reported in literature. We report the third case observed in a 43-year-old male, who presented with an 11-year history of right-sided hearing loss and a 1-year history of left-sided pulsatile tinnitus. A magnetic resonance imaging (MRI) showed a VS on the right side and computer tomography (CT) identified a Fisch type A1 paraganglioma on the left side. The VS was treated using a translabyrinthine approach and the MEP was kept under radiological observation for 1 year. Due to the growth of the MEP (Fisch type A2), it was treated with excision via a retroauricular approach. Our case was very challenging because there was a different and important pathology on each side, both carrying a risk of deafness as a consequence of the disease and/or the treatments.