Nephrogenic systemic fibrosis (NSF) is a dermatological fibrosing disease with potential systemic manifestations resulting in mild to severe symptoms including joint contractures. NSF can be lethal in a subset of cases. It has been recently observed that almost all patients with NSF have had severe renal insufficiency and have received prior administrations of gadolinium-based contrast agents (GBCA). This review will focus on the specific evidence from a retrospective study performed at our center in combination with the general experience available. Conclusions summarize our current understanding of NSF. Recommendations are provided for use of GBCA in clinical MRI practice. Conclusions and recommendations include that the risk of inducing NSF is related to higher-dose administrations of GBCA and that there are differences in risk related to differences in gadolinium-chelate formulation, with the highest relative risk associated with gadodiamide (Omniscan, Nycomed Amersham, GE Healthcare, Piscataway, N.J.).