Scleroderma or systemic sclerosis (SSc) is an autoimmune disease of the connective tissue, characterized by vascular abnormalities and progressive fibrosis of the skin and internal organs. Kidney, esophagus, heart and lung are most frequently involved. According to the extensive skin involvement and the internal organ injury, scleroderma is classified in limited and diffuse forms. Vascular injury is considered the first event in the pathogenesis of scleroderma. Vasculopathy primarily affects the microcirculation and the small vessels decreasing blood flow that results in chronic ischemia. Chronic vascular injury induces fibroblasts activation that leads to extensive fibrosis. Prevalence of renal involvement ranges from 10 to 40%. Its presentation can be very variable. The most serious renal complication is the scleroderma renal crisis associated or not with severe hypertension and acute renal failure. It is observed in 10% of the patients with scleroderma. Treatment with ACE-inhibitors modified significantly the prognosis of renal crisis. Other renal manifestations are chronic renal failure, nephrotic syndrome, ANCA-associated glomerulonephritis and isolated proteinuria.