Nephrogenic systemic fibrosis (NSF) is characterized by red skin areas or plaques that over several weeks successively develop to painful thickened skin with a 'woody' texture, resembling 'peau d'orange'. Starting at the extremities, it may spread to the trunk, and may progressively inhibit flexion of adjacent joints. In skin biopsies of affected areas, thickened collagen bundles, mucin deposition, and proliferation of fibroblasts and elastic fibers are seen. Originally described as nephrogenic fibrosing dermopathy (NFD) because of its primarily cutaneous manifestation, this entity was then named NSF because of systemic involvement of other organs like lungs, myocardium, or striated muscles. The pathogenesis of the disease is not yet known, but our observations suggest a close association between development of NSF and exposure to gadolinium-containing contrast agents, thereafter confirmed by other authors. Recently, gadolinium was demonstrated to be detectable in skin tissue samples of affected patients. In this short review, the development of NSF and its sequential association with the exposure to gadolinium-containing contrast agents is presented. The mechanisms likely to cause NFD/NSF are discussed.