Intracranial hemorrhage in patients with moyamoya disease is often caused by rupture of the associated aneurysms. Of these aneurysms, distal anterior choroidal artery (AChoA) aneurysms are rare. In patients with moyamoya disease, the AChoA constitutes collateral vessels and the aneurysm requires careful treatment strategy. However, reported cases of distal AChoA aneurysms include various procedures including conservative therapy, direct surgery, and endovascular therapy. Herein, we report a case of coil embolization of a distal AChoA aneurysm associated with moyamoya disease and discuss the treatment strategy. A 39-year-old female presented with severe headache and subsequent deep coma. Computed tomography (CT) revealed thick intraventricular hemorrhage, and three-dimensional CT angiography revealed a right distal AChoA aneurysm. Bilateral ventricular drainage was performed and subsequent ventriculoperitoneal (VP)shunt was performed. The persisting distal AChoA aneurysm was coil embolized without any complication. Rebleeding did not occur during the 1-year follow-up period. Endovascular treatment is effective for distal AChoA aneurysms associated with moyamoya disease to preserve collateral circulation.

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