Abstract: Moyamoya disease (MMD) is a chronic progressive cerebrovascular occlusive disease, and its ischemic symptoms, such as cerebral infarction and hemorrhage due to the angiorrhexis of the abnormal venous blood network of the skull base. The former may occur during childhood, whereas the latter may occur in adulthood. The continuous development of nuclear medical imaging technology has provided new methods and abundant information for determining the affected area, scope, and degree of the disease and for studying the cerebral blood flow reserves of patients. Thus, the technology is essential to the clinical treatment of MMD and evaluation of the efficacy of MMD treatment. The current paper reviews the progress in MMD research by nuclear medical imaging technology.