A case of pseudoxanthoma elasticum with multisystem involvement is described. Neurological complications, as reported in the literature, are reviewed. These include cerebrovascular insufficiency, multiple lacunar infarcts, aneurysms, subarachnoid and intracerebral hemorrhages, progressive intellectual deterioration, and psychic and mental disturbance which may be due to cortical atrophy. Seizures occur more frequently than in the general population. Hypertension and alteration of cerebral vessels are the two basic pathophysiological mechanisms responsible for the neurological complications of this disease.