BACKGROUND Fibromuscular dysplasia is an infrequent angiopathy that occurs more often in young women. It consists in a series of heterogeneous histological changes that finally lead to the narrowing of arteries. Clinical manifestations depend on the vessel involved, but high blood pressure (renal artery involvement) and stroke (carotid artery involvement) are the most common. Little has been published about this process in recent years. METHODS A 30-year-old female with extensive infarction of the right middle cerebral artery caused by obstruction of the internal carotid artery. Arteriographical examination revealed the dissection of the four supra-aortic trunks together with alterations to the renal artery. The patient's age and the involvement of the four cerebral arteries with irregular stenoses led us to consider a fibromuscular dysplasia (FMD) as the first diagnostic option. A conservative attitude was adopted towards the vascular lesions and therapy was carried out with a view to rehabilitating the neurological deficits. CONCLUSIONS Brain FMD can be asymptomatic and is often diagnosed by chance. The usual clinical presentation is a stenosis-related ischemic stroke, arterial obstruction or arterio-arterial thromboembolism. Arteriography or magnetic resonance angiography are useful for diagnosis; the involvement of different supra-aortic vessels is characteristic. Despite the good results shown by percutaneous transluminal angioplasty, there is still a need for prospective studies that determine the optimal treatment for this pathology.