The nephrogenic systemic fibrosis - referred to in the literature earlier as nephrogenic fibrotizing dermopathy - is a rare clinical entity that develops in patients with renal disease. Its development has been described mainly after the usage of a gadolinium-based MRI contrast medium in patients with impaired renal function, mainly in dialysed patients. The nephrogenic systemic fibrosis is characterized by fibrosis evolving in the distal part of the extremities, and in more severe cases involving the lung, the liver, heart and skeletal muscles. The disease may have a fast progression and fatal outcome if it involves multiple organ systems, in approximately 5% of the cases. Until now, no evidence-based therapy of nephrogenic systemic fibrosis is known, however, in single cases an amelioration has been described after renal transplantation and plasmapheresis, and remission has been described after extracorporal photopheresis.