A 50-year-old female presented with a rare fenestration of the proximal anterior cerebral artery (A
1 segment). This rare congenital anomaly was associated with an aneurysm at the proximal end of A
1 fenestration causing subarachnoid hemorrhage, and aplasia of the contralateral A
1 segment. Surgical treatment to clip the aneurysm resolved her symptoms. Hemodynamic stress at the arterial fenestration probably caused the aneurysm, possibly induced by aplasia of the contralateral A
1 segment.
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