OBJECTIVES: The objective of this case report was to describe a rare presentation of corkscrew cerebral angiopathy presenting as subarachnoid hemorrhage (SAH). METHODS: We present a young woman who presented with a thunderclap headache, found to have a nonaneurysmal SAH. RESULTS: Cerebral angiogram revealed corkscrew angiopathy in medium-sized vessels and multiple micro-occlusions with collateralization. No intracranial aneurysm was detected. Extensive workup for vasculitis and genetic causes for vasculopathy was unrevealing. The patient had no neurologic deficits, and her symptoms resolved. DISCUSSION: This is an extremely rare presentation of subarachnoid hemorrhage due to corkscrew angiopathy.