Objective To investigate the clinical features of acquired immunodeficiency syndrome (AIDS) with neurological complications. Methods Five with neurological complications out of 31 patients with confirmed AIDS in Shanghai area from 1992 to 2001 were analyzed. Results Of the five cases, there were one complicated with vacuolar myelopathy, one vacuolar myelopathy accompanied with dementia, one cerebral tuberculoma accompanied with dementia, one cerebral infarction accompanied with symptomatic epilepsia and trigeminal neuralgia, one polyneuropathy. Two of the vacuolar myelopathy are those showing first occurring manifestations. Conclusion AIDS patients are found at high risk of neurological complications, of which vacuolar myelopathy and AIDS related dementia are more common. If young patients who suddenly suffered from dementia, especially in patients accompanied with opportunistic infections, AIDS should be considered. In these cases serum HIV antibodies should be checked.