Objective To investigate the clinical neurological manifestations of Takayasu arteritis (TA). Methods A retrospective study was conducted with 63 consecutive TA cases admitted to Peking Union Medical College Hospital from January 2009 to May 2010. All the patients fulfilled the diagnostic criteria of TA by the American College of Rheumatology. Among the 63 TA patients, 27 with neurological manifestations were included in the present study. All the patients were evaluated using standardized neurological examination, sonography, computed tomography (CT) angiography, and cerebral CT or magnetic resonance imaging. Results Dizziness and visual disturbance were the most common symptoms, which occurred in 20 (74.1%) and 16 (59.3%) patients respectively. Another common symptom was headache, observed in 15 (55.6%) patients. Six (22.2%) patients had suffered from ischemic stroke; 7 (25.9%) patients had epileptic seizures. Two (7.4%) patients were diagnosed as reversible posterior encephalopathy syndrome (RPES) based on typical clinical and imaging manifestations. Conclusions Neurological manifestations are common symptoms in TA patients in the chronic phase, including dizziness, visual disturbance, headache, ischemic stroke, seizures, and some unusual ones such as RPES. We suggested RPES be included into the differential diagnosis of acute neurological changes in TA. Objective To investigate the clinical neurological manifestations of Takayasu arteritis (TA). Methods A retrospective study was conducted with 63 consecutive TA cases admitted to Peking Union Medical College Hospital from January 2009 to May 2010. All the patients fulfilled the diagnostic criteria of TA by the Among the 63 TA patients, 27 with neurological manifestations were included in the present study. All the patients were evaluated using standardized neurological examination, sonography, computed tomography (CT) angiography, and cerebral CT or magnetic resonance imaging. Another common symptom was headache, observed in 15 (55.6%) patients. Six (22.2%) patients had 16 (59.2%) patients. Six (22.2% 7 (25.9%) patients had epileptic seizures. Two (7.4%) patients were diagnosed as reversible posterior encephalopathy syndrome (RPES) based on typical clinical and imaging manifestations. Conclusions Neurological manifestations are common symptoms in TA patients in the chronic phase, including dizziness, visual disturbance, headache, ischemic stroke, seizures, and some extraordinary ones such as RPES. We suggested RPES be included into the differential diagnosis of acute neurological changes in TA.