A 69-year-old man presented with a cholesteatoma in the right mastoid process and a vestibular schwannoma at the left internal acoustic meatus. Cholesteatoma co-existing with a vestibular schwannoma has not been documented previously in the contemporary literature. The clinical dilemma in the management of his progressive bilateral hearing loss is discussed. He presented with dizziness and bilateral hearing loss worse on the right side. Pressure over the mastoid process elicited vertigo and nystagmus. He had no history of previous operation or infection in the ear canal. Audiograms confirmed high-tone hearing loss. Radiological investigations revealed a symptomatic cholesteatoma on the right side and an incidental vestibular schwannoma on the left. We have elected to manage both lesions conservatively. Bilateral cholesteatoma and bilateral vestibular schwannomas have been previously reported. Co-existing lesions, as in our patient have, however, not been reported previously. The management options of his hearing loss are discussed.