Objective : We examined the incidence of fenestration in the proximal segment of the anterior cerebral artery[ACA] and reviewed its clinical significance. Methods : Cerebral angiography was performed in 843 patients and magnetic resonance angiography[MRA] in 1,787; all patients had, or were suspected to have, cerebrovascular disease. We subsequently reviewed the angiography and MRA data. Results : Fourteen patients [six men and eight women, $35{sim}81$ years of age, median age = 67 years] had proximal ACA fenestrations. Three of the 843 patients [0.36%] undergoing cerebral angiography had fenestrations of proximal ACA as did eleven of the 1,787 MRA patients [0.62%]. Seven fenestrations were located on the right side and seven fenestrations were located on the left side. The fenestrated ACA in one patient was associated with a saccular aneurysm at its proximal end. None of the 120 patients who underwent both conventional cerebral angiography and cranial MRA showed fenestration of proximal ACA. Conclusion : Although fenestration of ACA has little clinical significance, knowledge and recognition of this condition is important in the interpretation of cerebral images, especially during neurosurgical procedures.