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Introduction. Azygos anterior cerebral artery is a rare variant of the anterior segment of the circle of Willis caused by an unusual fusion of the normally paired A2 segments of the anterior cerebral artery (ACA). Despite its rare occurrence, it is associated with various vascular and structural cerebral abnormalities, particularly berry aneurysms. Aim. We aimed to present a case of a 41-year-old female patient who presented to our neurology department with complaints of headache. Description of the case. She had a positive paternal history of aneurysmal subarachnoid hemorrhage. Magnetic resonance angiography (MRA) of her brain revealed an azygos ACA (bifurcating into two pericallosal arteries) which was associated with a saccular aneurysm at its bifurcation point. She was referred to the interventional radiology department for preventive endovascular treatment. Conclusion. Azygos ACA carries a high risk of aneurysm development and its occlusion can potentially compromise blood supply to both cerebral hemispheres. It is therefore crucial for clinicians to be aware of its significance and to report its presence in angiographic studies.
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