Nephrogenic systemic fibrosis (NSF) is a progressive, potentially fatal systemic multiorgan fibrosing disease related to exposure of patients with advanced renal failure to the gadolinium-based contrast agents (GBCAs) used in magnetic resonance imaging. Because of this relationship between nephrogenic systemic fibrosis and gadolinium-based contrast agents, the U.S. Food and Drug Administration currently warns against using gadolinium-based contrast agents in patients with a glomerular filtration rate less than 30 ml per minute per 1.73 m2, or any acute kidney injury related to the hepatorenal syndrome or perioperative liver transplantation. Linear non-ionic GBCAs that are more prone to release free gadolinium are the more likely to cause NSF. The mechanism for NSF is not fully understood, yet risk factors have been described. As there is no established therapy for NSF the prevention of exposure to gadolinium is crucial in high risk patients.