Nephrogenic systemic fibrosis is a debilitating disease occurring exclusively in patients with severe renal failure. Originally it was described as nephrogenic fibrosing dermopathy. The pathogenesis of the disease is not yet known, but the observations suggest a close association with the exposure to gadolinium-containing contrast agents. These agents cause systemic fibrosis by releasing free gadolinium into tissues. International commissions in drug control recommend avoiding gadolinium based contrast agents ion patients with GFR <30 ml/min. The prevention is the best treatment because the observations are series with limited patients, or a case-report communication. So, we report a case with a fatal evolution, the first case in Spain, with a multisystemic involvement showed in the autopsy. The patient had repeated exposure to gadodiamide; it increases the possibility of the systemic fibrosis. We review the recent literature of the nephrogenic systemic fibrosis.