Systemic sclerosis (SSc) is an autoimmune disease of connective tissue characterized by fibrosis of the skin and internal organs, vascular damage, and immunological changes. One of the most severe complications is renal involvement, particularly scleroderma renal crisis (SRC). Renal crisis is one of the most serious complications of SSc and often occurs in diffuse SSc. This article discusses the pathogenesis, clinical manifestations, diagnostic methods, and modern approaches to treatment of renal involvement in SSc.