Fibromuscular dysplasia (FMD) is a rare disease with a typical clinical manifestations. This article will describe the clinical features of a FMD case with se-vere headache misdiagnosed as cerebral artery dissection (CAD). Treatment remedies: we collected the clinical data of a patient with severe headache and performed brain MRI (magnetic resonance imaging) and cerebral arteriovenous MRA (magnetic resonance angiography). The preliminary diagnosis was considered as right ver-tebral artery dissection, and then cerebral DSA (digital subtraction angiography) was performed for further di-agnosis. Post treatment evaluating: cerebral angiogra-phy showed that the vessel wall was stiff, with multiple (insect phagocytic)-like changes, and severe stenosis of local long segments. Some vessels showed “double lu-men sign”, which was consistent with the performance of FMD. After anticoagulant treatment, the patient's con-dition was improved. Conclusions: for severe headache, in addition to common diseases, the possibility of FMD should also be paid great attention. Early cerebrovascu-lar screening is conducive to early diagnosis and timely treatment.