Gadolinium-based contrast agents (GBCAs) have been suggested as off-label alternatives to iodine-based contrast agents for fluoroscopic imaging during interventional pain procedures. We report a case of accidental intrathecal administration of a GBCA during a neuraxial interventional pain procedure leading to acute gadolinium neurotoxicity, which resulted in encephalopathy and ultimately death. To our knowledge, it is the first published case of fatal intrathecal gadolinium-induced encephalopathy and the first published case of intrathecal gadoteridol causing serious neurologic complications. In addition, the case presented here is placed in context with an associated comprehensive, evidence-based review of the use of gadolinium in interventional pain procedures, addressing gadolinium chemistry and pharmacologic properties, neurotoxicity and radiology. Physicians must be aware that gadolinium poses a significant risk of acute neurotoxicity even in small doses. Until further safety research is performed, GBCAs should not be considered a safe alternative for use in neuraxial interventional spine procedures when there is a risk of inadvertent intrathecal administration.