The aim of this article is to review the published cases of nephrogenic systemic fibrosis (NSF) in Japan. The Japanese medical literature database and MedLine were searched using the keywords NSF and nephrogenic fibrosing dermopathy (January 2000 to March 2009). Reports in peer-reviewed journals and meeting abstracts were included, and cases with biopsy confirmation were selected. 14 biopsy-verified NSF cases were found. In seven of eight patients reported after the association between gadolinium-based contrast agent (GBCA) and NSF was proposed, GBCA administration was documented: five received only gadodiamide; two received both gadodiamide and gadopentetate dimeglumine. In four cases, the amounts of contrast agent were registered: two received only a single dose (0.1 mmol kg(-1) body weight) whereas the other two received 7-15 ml (the body weight was not disclosed) for each MR examination. Five patients had multiple injections of GBCA before NSF developed. Except for one patient in whom renal assessment was not reported, none of the patients had an estimated glomerular filtration rate >30 ml min(-1) 1.73 m(-2) and all received dialysis. 5 of the 8 patients (63%) in whom GBCA exposure was confirmed were treated with peritoneal dialysis. Skin lesion of the lower extremity was the first symptom in 12 patients (86%), whereas 2 patients had primarily symptoms from the upper extremity. In three cases, GBCA was administered even after onset of the NSF symptoms because of the physicians' lack of knowledge about the possible association between GBCA and NSF. NSF is found among Japanese end-stage renal failure patients even after examinations using a single dose.