Fibromuscular dysplasia (FMD) was first described in the medical literature in 1938. A 5-year-old boy with hypertension and an ectopic right kidney was cured after a right-sided nephrectomy. On histology, his right renal artery was partially obstructed by an intraluminal smooth muscle mass [1]. Further case reports and series were published over time, describing renal [2] and carotid involvement [3], pathological classifications [4] and treatments [5, 6]. This century, the focus has moved from case reports and series to national and international registry data [7–10] and from pathology and histology to the discovery of unique biomarkers [11], to try to learn more about the aetiology and the natural history of this still poorly understood disease.