A review of nephrogenic systemic fibrosis (NSF), a severe disorder described in patients with renal disease, appears as timely as the pathogenic role of gadolinium-based contrast agents (GdBCAs) has now been convincingly demonstrated, leading to preventive guidelines.The link between NSF and gadolinium (Gd) exposure has been reinforced by the identification of Gd in the skin of patients who denied having been exposed to GdBCA and nevertheless developed the disease, suggesting that the few cases reported in the absence of Gd exposure could be explained by recall bias. Experimental studies have shown that repeated infusions of gadodiamide to rats reproduced the disease. Exposure of monocyte cultures to Gd led to secretion of numerous cytokines and growth factors which could induce fibrosis. Recent studies confirmed the association with infusion of the less stable linear GdBCAs, especially gadodiamide, and the role of high dosing of GdBCA, as performed in magnetic resonance angiography. Differences in the choice of GdBCA and angiography technique might explain wide reporting variations across centres.NSF appears as a iatrogenic disease linked to the infusion of GdBCA. Its incidence can be reduced by avoiding exposure of patients with stages 4 and 5 chronic kidney disease to GdBCA.