PURPOSE OF REVIEW: The aim of this article is to outline the history of nephrogenic systemic fibrosis, a new and serious disease of patients with renal failure, and to give an update on its aetiology and prevalence. RECENT FINDINGS: Epidemiological and histochemical studies demonstrated that gadolinium-containing contrast agents used for magnetic resonance imaging have an essential causative role in most, if not all, cases of nephrogenic systemic fibrosis. One particular agent, gadodiamide, caused the majority of cases, but gadopentetate dimeglumine has also been implicated in several cases. Increasingly poor renal function, aberrations in calcium-phosphate metabolism and erythropoietin treatment seem to increase the risk of the disease and its severity. Up to 25-30% of patients with renal failure exposed to gadolinium-based contrast agents may develop nephrogenic systemic disease. The figure varies with type of gadolinium-based contrast agent and patient characteristics. There is no established curative treatment of the disease, but rapid recovery of renal function or hastened kidney transplantation may be beneficial. SUMMARY: It is mandatory to avoid exposing patients with renal failure to gadodiamide and gadopentetate dimeglumine. Other linear gadolinium-based contrast agents should be used with great caution. It is premature to conclude whether cyclic gadolinium-based contrast agents are totally safe in renal failure.