Background: Moyamoya disease (MMD) is a rare cerebrovascular disease characterized by bilateral stenosis starting at the supraclinoid internal carotid artery (ICA), with the development of a collateral network of vessels. It is an established cause of stroke in the pediatric age group. Despite its increasing prevalence in various parts of the world, it remains largely underrecognized in the Middle East, particularly in Iraq. This is the first case of MMD in an Iraqi patient undergoing surgery. Case description: A 12-year-old boy presents with a 3-months history of progressive behavioural changes. MRI revealed diffuse infarcts of different ages. MRA and CT angiography revealed extensive asymmetrical steno-occlusive changes of the supraclinoid ICAs extending into the anterior and middle cerebral arteries, with the development of a collateral network in the basal ganglia. Indirect revascularization of the right side by encephaloduroarteriomyosynangiosis (EDAMS) was performed. The clinical status of the patient improved during the follow-up and the MRA showed a re-establishment of the blood flow to the MCA. Conclusion: MMD should be recognized as a cause of stroke or recurrent TIAs in the Iraqi population, particularly in pediatric patients. EDAMS is an effective revascularization procedure with good results in pediatric patients.