52 year old male patient was admitted to the stroke unit with severe headache, nausea, vomiting and imbalance. The patient has suffered from severe headaches, hypertension and dizziness during the past 5 years. During the neurological examination subarachnoid hemorrhage (SAH) was suspected and observed on brain CT. Cerebrovascular risk factors except hypertension were excluded. Noninvasive investigations, such as Duplex ultrasound and MRA were initially performed for evaluation of the cause of SAH. The results of investigations were suggestive of fibromuscular dysplasia (FMD), therefore catheter-based angiography was done to support the diagnosis. Typical “string of beads” pattern of the left VA and pseudoaneurysm of the left vertebral artery consistent with FMD were detected, causing the SAH. Clinical features and specific pattern of FMD on investigations despite the male gender were supportive of the diagnosis of FMD as a rare cause of stroke