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Segmental arterial mediolysis (SAM) is a rare arterial pathology and can cause rupture or dissection of the intracranial arterial wall. The etiology is unveiled, but vasospastic stimuli such as migraine are considered as a possible cause of SAM. We present the first case of subarachnoid hemorrhage (SAH) due to SAM associated with Crohn's disease and migraine, and discuss the possible contribution of Crohn's disease to the development of SAM besides migraine.