Objective: To present the association of Ménière’s disease (MD) in one ear and vestibular schwannoma (VS) in the contralateral ear as a rare clinical entity, and discuss management options.Methods: A retrospective chart review of all patients diagnosed with MD over a ten year period was conducted for patients that met a definite diagnosis of MD in the ear contralateral to the ear with VS diagnosis, based on the 1995 MD criteria set by American Academy of Otolaryngology—Head and Neck Surgery.Results: Of 974 patients that met the inclusion criteria for a diagnosis of VS, five patients had a diagnosis of contralateral MD. The age range was 32-55 years. The size of VS ranged from 3-25 mm. All patients had the MD component managed with an aggressive medical protocol, and short courses of prednisone. For VS, four patients underwent surgery (three translabyrinthine, one retrosigmoid), and one patient opted for stereotactic radiation.Conclusion: The association of MD in one ear and VS in the contralateral ear represents a rare clinical scenario. Bilateral deafness and/or bilateral labyrinthine hypofunction are interventional risks that can lead to severe patient incapacitation. Tumor size, brainstem compression, tumor growth, residual functional hearing, and the degree of disability of MD vertiginous symptoms are important considerations in this management algorithm.