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Mitchell W. Couldwell, Samuel Cheshier, Philipp Taussky, Vance Mortimer, and William T. Couldwell

Moyamoya is an uncommon disease that presents with stenoocclusion of the major vasculature at the base of the brain and associated collateral vessel formation. Many pediatric patients with moyamoya present with transient ischemic attacks or complete occlusions. The authors report the case of a 9-year-old girl who presented with posterior fossa hemorrhage and was treated with an emergency suboccipital craniotomy for evacuation. After emergency surgery, an angiogram was performed, and the patient was diagnosed with moyamoya disease. Six months later, the patient was treated for moyamoya using direct and indirect revascularization; after surgery there was excellent vascularization in both regions of the bypass and no further progression of moyamoya changes. This case illustrates a rare example of intracerebral hemorrhage associated with moyamoya changes in the posterior vascularization in a pediatric patient and subsequent use of direct and indirect revascularization to reduce the risk of future hemorrhage and moyamoya progression.