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Copyright © 2020 Asian Journal of Neurosurgery.Moyamoya disease (MMD) is an incompletely understood malady that affects many age groups, primarily in a bimodal age distribution. We present a patient with the association of type 1 diabetes mellitus (type 1 DM) and MMD followed by a review of the existing literature. We found five papers that describe this association, in the form of one case report, one case series, and three retrospective reviews. Despite a poor understanding of the underlying pathophysiology, a clear association betwe