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Moyamoya disease (MMD) is an idiopathic progressive steno-occlusive disease in the internal carotid artery (ICA) bifurcation. In rare circumstances, transdural anastomotic aneurysm (TAA) could develop during the progression of MMD. We present an illustrative case of TAA in association with MMD. To further explore this rare entity, a comprehensive literature review was also conducted. Our illustrative patient experienced spontaneous remission of the aneurysm during follow-up. By literature review, 12 patients with 13 TAAs, including our