Nephrogenic systemic fibrosis is a highly debilitating and emotionally depressing disorder. So far, it has exclusively occurred in patients suffering from acute or chronic renal failure. Most patients had been exposed to gadolinium- based contrast agents. Early symptoms include pain, swelling and pruritus, predominantly of the extremities. Fibrosis of the skin and other organs develops in the further course of the disease. The diagnosis is made based on the synopsis of medical history, clinical and dermatohistological findings. To avoid the application of gadolinium-based contrast agents in patients with highly impaired renal function is the best measure to prevent the disease. An effective therapy has not yet been established. Possible therapies, which have been successful in individual cases, comprise, among others, a quick restoration of renal function, physiotherapy, and extracorporeal photopheresis.