Moyamoya disease (MMD) is caused by stenosis or occlusion of internal carotid artery in brain, thereby reducing its blood supply. To augment blood flow, brain develops abnormal anastomotic vessels with deranged carbon dioxide reactivity and tendency to bleed. Moyamoya syndrome (MMS) is the name given to MMD when the latter results from secondary to some associated disease. Occurrence of MMS secondary to sickle cell anemia (SCA) presents unique challenges to neuroanesthesiologists. Management of various physiological parameters for cerebral revascularization surgery for MMD under general anesthesia necessitates vigilant and balanced control of various physiological variables, as the manipulation of a particular physiological variable for one pathology may adversely impact the same physiological variable for the associated disease, which will result in poor outcome of the patient. Therefore, optimum outcome of MMS is determined by a watchful balancing of various physiological parameters under anesthesia.