Nephrogenic systemic fibrosis (NSF), also known as nephrogenic fibrosing dermopathy, is a scleroderma-like disease of the skin observed in patients with renal insufficiency. Since its recognition in 1997, relatively little has been published in the nephrology literature, despite indications that the disorder is becoming increasingly recognized in the United States and abroad. As nephrology professionals may be the first to encounter newly developing cases of NSF, it becomes imperative that they be able to recognize the symptoms and comorbidities and initiate tests and treatment. To date, the cause of NSF has not been established. Recent observations indicate that specific cells normally involved in wound repair and tissue remodeling may be aberrantly recruited to the skin and soft tissues of NSF patients.