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Department of Neurosurgery. Selçuk University Medical School. Konya, Turkey A case of saccular aneurysm of the azygos anterior cerebral artery is reported. Aneurysms of the azygos anterior cerebral artery are extremely rare. Clinical, radiological and operative findings are described in detail and the pertinent literature is reviewed. Keywords : Anterior cerebral artery. Azygos anterior cerebral artery. Saccular aneurysm