We reviewed charts of patients seen at Mayo Clinic Rochester and Mayo Clinic Jacksonville to determine the importance and frequency of optic disk swelling in patients with peripheral neuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS syndrome). A computer search identified 148 patients who were classified as having a monoclonal gammopathy and peripheral neuropathy. A review of the 148 charts disclosed that 11 patients had POEMS syndrome. Eight of these 11 patients had optic disk swelling identified by an ophthalmologist. In all eight patients, optic disk swelling was either the initial sign or was present the first time we saw the patient. Optic disk swelling may be an early sign in POEMS syndrome.