Nephrogenic systemic fibrosis (NSF) is a systemic disease, recently described in patients with advanced chronic kidney disease (CKD), characterized by progressive scleromyxedema-like fibrotic involvement mainly of the skin. We describe the case of a 66-year-old woman on chronic hemodialysis for end-stage renal failure, also affected by hypothyroidism, secondary hyperparathyroidism and occluding arteriopathy, for which she underwent a contrast-enhanced magnetic resonance angiography of the lower limbs in February 2007. One month later, she began complaining of progressive, painful distal lower limb stiffness, which subsequently spread to all four limbs and to the whole trunk. A deep-skin biopsy, taken from an affected area, showed gadolinium deposits. The case reported is, to best of our knowledge, the first Italian case of NSF. This diagnosis should be considered with care in CKD patients with a recent exposure to a gadolinium-based contrast agent, complaining of limb stiffness, especially in the presence of risk factors.