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Vertigo and imbalance are believed to be rare manifestations of skull base neoplasms. Patients with skull base neoplasms can present with vague otolaryngological complaints, including diplopia, facial numbness, facial weakness, hearing loss, tinnitus, hoarseness, headache, and otalgia. Physical examination of these patients can sometimes reveal paralysis or paresis of cranial nerves. Magnetic resonance imaging (MRI) is the gold standard for evaluation of cranial nerve involvement in skull base diseases. Vertigo and imbalance can be manifestations of a neuropathy or lesion within the vestibular system and may be subtle or overlooked findings in patients with skull base diseases. The purpose of this article is to review the clinical manifestations of patients presenting with vertigo and imbalance who were found to have skull base neoplasms. We will also highlight the importance of MRI in diagnosis and management of these patients.