BackgroundRete mirabile of the cerebral artery is a rare anomaly, with most previous cases occurring in the middle cerebral artery or internal carotid artery. Here, we present the first report of unilateral rete mirabile in multiple intracranial arteries with ipsilateral internal carotid artery agenesis.Case presentationA 64-year-old Japanese woman was brought to the emergency department of our hospital in a deep coma. Computed tomography of the head showed severe intraventricular hemorrhage with subarachnoid hemorrhage. Computed tomography angiography showed not only congenital left internal carotid artery agenesis, but also rete mirabile of the left posterior communicating artery, the left posterior cerebral artery, and the left anterior cerebral artery. This unilateral vessel anomaly complex may have contributed to the formation of a peripheral aneurysm arising from a perforating branch of the pericallosal artery, which ruptured. The patient underwent urgent bilateral external ventricular drainage, but deteriorated and was declared brain dead.ConclusionsWe report the first case of unilateral rete mirabile in multiple intracranial arteries. Because the cerebral arteries in patients with rete mirabile may be vulnerable, close attention should be paid to the development of cerebral aneurysms.