Degos' disease, a rare multisystem vasculopathy of unknown etiology, only occasionally involves the nervous system. We report the Mayo Clinic experience of the neurologic features of Degos' disease in a series of 15 patients. All 15 patients had the typical skin lesions of Degos' disease, confirmed by skin biopsy. Ten patients developed neurologic manifestations including fatal hemorrhagic or ischemic strokes (n=5), disabling polyradiculoneuropathy (n=1), and nonspecific neurologic symptoms without objective findings (n=4). Results of laboratory tests varied but none were pathognomonic of the disease. Long-term follow-up revealed death in six patients; nine patients were nearly asymptomatic. Immunosuppressive and antiplatelet agents were not of benefit. CNS infarcts and hemorrhages with intravascular thrombi, but without evidence of vasculitis, were characterized features at autopsy.