Systemic sclerosis is a rare connective tissue disorder, potentially affecting several internal organs. Renal disease is seen in 10-40% of the cases, while autopsy studies have shown interstitial renal changes in as many as 80%. Renal involvement is clinically divided into two separate forms; chronic and acute nephritis. Acute nephritis in a patient suffering from systemic sclerosis is termed renal chrisis, and is associated with high morbidity and mortality. Renal crisis in systemic sclerosis is discussed based on a case report and a review of the literature. Treatment with ACE-inhibitors has dramatically improved the prognosis of this complication. Careful surveillance of patients at risk, especially patients with the diffuse type of systemic sclerosis and showing antibodies against RNA-polymerase, as well as early treatment is considered important for the outcome.