Nephrogenic systemic fibrosis (NSF) is a rare systemic fibrosing disorder that primarily affects the skin and the subcutaneous structures. Also, there are reports of involvement of deeper structures and organs in the human body, but the confirmation of systemic involvement is complicated by overlap of other disease processes that occur in patients with severe renal impairment. The disorder leads to significant disability and is an important contributing factor of death. Virtually all patients who developed NSF suffered from endstage renal disease (ESRD) or severe chronic kidney disease (CKD) or from an acute acquired kidney injury (AKI). There is an increase in evidence that a causal relation between gadolinium-based contrast agents (Gd-CA) and NSF is probable. Therefore, advanced kidney injury and the exposure to Gd-CA are regarded as prerequisites to develop NSF. Overall, the prognosis is poor and there is no established therapy that shows a consistent benefit. The purpose of this review is to discuss the clinical spectrum of the disease. The clinical presentation, role of co-morbidity in disease development and manifestation, time course, prognosis, outcome, and epidemiological aspects are especially reviewed. J. Magn. Reson. Imaging 2009;30:1289-1297. (c) 2009 Wiley-Liss, Inc.