Nephrogenic systemic fibrosis (NSF) is a systemic disease that affects the skin and other tissues in patients with renal insufficiency and exposure to gadolinium-containing contrast. A 55-year-old woman with end-stage renal disease on hemodialysis was consulted for progressive general weakness. After she had undergone multiple MRIs with gadolinium-containing contrast media, muscle weakness and skin lesions were developed. Her skin and muscle biopsy specimens showed CD34+ fibroblast entrapping collagen bundles. There are few reports of NSF with myopathy.