Neurologic manifestations/comp/ictions of systemic scleroderma have received less attention in the literature than cardiac, pulmonary or gastrointestinal manifestations. We describe briefly three autopsied and proven cases of progressive systemic sclerosis in which there had developed prominent neurologic symptoms and signs, and discuss the relationship of those symptoms and signs with progressive systemic sclerosis. A review of neurologic complications is presented specifically to direct the attention of physicians to such symptoms which may later result in increased reporting of neurologic symptoms and signs. Already there is increased awareness due to reports of conditions such as trigeminal neuropathy and myopathy in more recent years. Cerebral, and specifically cerebrovascular, manifestations may be recognized more frequently in the future. A brief note of etiopathology and treatment is also mentioned.