Chronic inflammatory demyelinating polyneuropathy (CIDP) has occasionally been associated with clinical or laboratory evidence (magnetic resonance imaging,[MRI], visual evoked response, and brainstem auditory evoked response [BAER] of cranial neuropathy. In most cases, the relationship of cranial nerve involvement to CIDP remains unclear. A 45-year-old woman noted foot numbness, limb weakness, gait and postural instability, and oscillopsia. An IgG kappa monoclonal gammopathy of undetermined significance was found. Bilateral vestibulopathy was documented by clinical examination, bithermal calorics, rotary chair testing, BAERs, and dynamic posturography. MRI with gadolinium demonstrated enhancement of cranial nerve VIII bilaterally. Over the next 6 years, the patients's relapsing and remitting course of CIDP and vestibulopathy was assessed by quantitative muscle and vestibular function testing (clinically and neurophysiologically), and dynamic visual acuity. There was a striking synchronization between her CIDP and vestibulopathy with respect to clinical course including relapses and responses to immune therapy. The response to therapy, and evidence derived from clinical and laboratory investigations, suggest that the vestibular dysfunction was immune mediated.