Objective：To evaluate the endothelial functions and autoregulation capacity of cerebral blood flow in patients with Fabry disease. Methods：Brachial artery vasodilation was assessed in 8 patients with Fabry disease and 14 healthy controls by means of flow-mediated dilation (FMD) and Nitroglycerin-mediated dilation (NMD). Cerebrovascular reactivity was calculated in terms of breathholding index (BHI) and vascular motor reactivity (VMR) by TCD-CO2 test in 4 patients and 14 healthy controls. Results：Compared with the controls, brachial artery vasodilation experiment showed no difference (the patients: FMD 15.94%±5.03% and NMD 23.92%±7.23%, the controls: FMD 14.57%±5.84% and NMD 22.64%±6.96%), there was no relationship between FMD or NMD and the age, course of disease, MSSI or enzyme activity. In respect of cerebrovascular autoregulation capacity, there was no difference in anterior circulation, while cerebrovascular reactivity tended to be impaired in posterior circulation. Conclusion：Endothelial function showed no decline in patients with Fabry disease, but cerebrovascular autoregulation capacity tended to be impaired in posterior circulation.