Systemic sclerosis (SSc) is a multisystem disease whose clinical manifestations result from inflammation, vascular injury and obliteration, and cutaneous and visceral fibrosis. Scleroderma renal crisis (SRC) occurs in 5% of patients with particullary diffuse form of SSc. It is characterized by malignant hypertension and oligo/ anuric acute renal failure. SRC was once a uniformly fatal complication of SSc. The prognosis of SRC has significantly improved with the introduction of angiotensin-converting enzyme inhibitors (ACEi) as treatment. The treatment of SRC relies on tight control of blood pressure and aggressive treatment with ACEi, if needed in combination with other types of antihypertensive drugs.