A 34-year-old woman with terminal renal insufficiency presented with thickening and hardening of the skin of the extremities, resulting in contractures of the joints and severe disability. Serology revealed no signs of autoimmune disease, apart from a positive result for antinuclear antibodies. Histological evaluation of a skin biopsy showed marked fibrosis of the entire dermis, extending into the subcutaneous fat, with CD34-positive fibrocytes. The clinical features resembled a recently reported new disorder: nephrogenic systemic fibrosis (NSF). This disease causes fibrotic changes in the skin and other organs in patients with (pre)terminal renal insufficiency. The cause of the disease is still unknown, although there are strong indications that exposure to gadolinium-containing contrast agents plays a role in the pathogenesis. To prevent more patients from developing NSF, the Dutch Medicines Evaluation Board has changed the clinical indications for the use of gadolinium-containing contrast agents in patients with kidney disease: gadodiamide (Omniscan) and gadopentetate dimeglumine (Magnevist) may not be used in patients with severe renal failure or patients that will undergo or have already undergone liver transplantation. Caution is advised in patients with moderate renal insufficiency; this also applies to the other registered gadolinium-containing contrast agents.