Charcot-Marie-Tooth (CMT) disease is a heterogeneous group of genetically mediated peripheral sensorimotor neuropathies and comprises the most common hereditary peripheral neuropathy. Different subtypes have different modes of transmission and different combinations of axonal and/or demyelinating features. CMT can cause disequilibrium by virtue of its sensorimotor deficits, but some subtypes also have features of vestibular weakness; less commonly reported features include cerebellar and autonomic deficits. CMT can also cause sensorineural hearing loss that is usually, but not always, approximately symmetrical and gradually progressive. Otovestibular tests may reveal vestibular weakness (such as on video head impulse testing, caloric testing, or cervical vestibular evoked myogenic potentials). If a CMT patient is referred to a vestibular medicine clinic, it is medically reasonable to undertake a screening otovestibular workup to seek more common causes of disequilibrium, before concluding that CMT is the exclusive cause of disequilibrium. There is not yet any curative or arrestive therapy. However, in CMT patients found to have vestibular weakness, a trial of vestibular rehabilitation therapy is reasonable.