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Fibromuscular dysplasia (FMD) is a rare nonatherosclerotic, noninflammatory vascular disease that most often affects the renal and carotid arteries and is the second most frequent cause of renal artery stenosis. The symptomatology is dominated by arterial hypertension due to the frequent involvement of the renal arteries and depends on the location of the lesions. Most of the cases are middle-aged women of Caucasian origin. There are two subtypes based on angiographic aspect: multifocal FMD (80% of the cases), and focal FMD (rarer with a more balanced sex ratio). Angioplasty of the renal arteries is generally disappointing, with a less-than-50% success rate when it comes to curing hypertension. It appears necessary to improve our knowledge of FMD and to optimize the selection of eligible patients for revascularization by way of transdisciplinary collegial therapeutic decisions.