Systemic sclerosis (SSc) is a kind of autoimmune disease characterized by inflammatory and endothelial dysfunction. Asymmetric dimethylarginine (ADMA), as an endogenous nitric oxide synthase inhibitor, can cause or contribute to the inflammatory syndrome and endothelial dysfunction. Recently, increased ADMA levels have been demonstrated in SSc, revealing that ADMA might play an important role for the associated manifestations of SSc. Besides, ADMA may play a significant role in the level of NO, which is produced by arginine. In the review, we discuss the role of arginine and ADMA in patients with SSc.