Cerebral aneurysms are occasionally associated with anomalies of the cerebral arteries. Most reports on anomalies of the anterior cerebral artery have been concerned with hypoplasia, fenestration and the infra-optic course of the A1, variant A1 perforators or Heubner's artery, multi-channeled anterior communicating artery, and azygos anterior cerebral artery. Distal anterior cerebral artery (ACA) aneurysms are known to have a poor clinical course and prognosis compared to other supratentorial aneurysms. The presence of the unpaired, distal, postcommunicating (A2) segment of the ACA is very rare in adults. We describe a patient with a ruptured aneurysm arising from the proximal end of the azygos ACA, first surgically treated with clipping and then with endovascular coiling. A 37-year-old woman at 34 weeks' gestation was transferred to our emergency room with sudden onset of severe headache and vomiting. Computed tomography (CT) revealed subarachnoid hemorrhage in the basal cisterna and the sylvian and interhemispheric fissures. Cerebral angiography showed an azygos ACA, a saccular aneurysm at the junction of the azygos ACA and the right A1 segment. A right fronto-temporal craniotomy was performed in the day of admission, and the neck of the aneurysm was clipped. One year later, an angiographic control examination revealed a regrowth of the aneurysm. The patient underwent endovascular treatment with coiling. Aneurysms of the azygos ACA are rare and their pathogenesis and course are still a matter of discussion. Developmental abnormalities or dynamic vessel wall stresses can explain the high incidence of aneurysms in these cases. The association of a rare anatomical variant with an aneurysm in the same location may suggest an embryogenesis alteration in the Willis circulation.