Summary: Fibromuscular dysplasia (FMD) is idiopathic, segmental, non- -inflammatory, non-atherosclerotic disease affecting small and medium-sized arteries. FMD causes artery stenosis, although it may also lead to aneurysm, dissection or arterial tortuosity. There are two types of angiographic appearance of FMD: multifocal (presenting as “string of beats”) and focal. FMD affects mainly young and middle-aged women and occurs in renal arteries, but it may be found in any arterial bed. This article provides a detailed overview of current knowledge of clinical characteristics, diagnostics, complications and treatment of patients with FMD.