Nephrogenic systemic fibrosis (NSF) is a novel disease entity described over the past 10 years. NSF is a progressive systemic fibrosing disorder that occurs arguably exclusively in patients with impaired renal function who have been exposed to gadolinium-containing contrast agents. As no single clinical or histopathologic finding is diagnostic of NSF, a careful review of the cumulative characteristics of each case is essential in making a correct diagnosis. The spectrum of histologic variants of NSF continues to expand, including a report of NSF mimicking erythema nodosum and several case reports of NSF with giant cells and calcification. We report an additional case of NSF with the uncommon pathologic features of granulomatous and lymphocytic inflammation in the fibrous septae similar to erythema nodosum.