Unilateral presentation of vestibular schwannoma with concurrent chronic middle ear disease or cholesteatoma is rare. We report a case series of patients with ipsilateral chronic middle ear disease and vestibular schwannoma, which were simultaneously removed via the enlarged translabyrinthine approach. All tumors were near-totally removed, and middle ear disease was completely excised; there were no major postoperative complications such as cerebrospinal fluid leakage, cholesteatoma recurrence, or meningitis. If hearing preservation of the affected ear is not necessary, simultaneous surgical removal of both pathologies is more convenient than staged treatment. The enlarged translabyrinthine approach can achieve complete treatment with one corridor.