Kidney involvement in systemic sclerosis (SSc) is primarily manifested by scleroderma renal crisis (SRC). More than 30 years ago, it was the main cause of death in these patients. The use of AC inhibitors has modified the prognosis and nowadays SRC has become a much more easily treatable complication of SSc. Furthermore, although there are still many patients who do not survive this complication, the early diagnosis and prompt therapy of SRC can have an excellent outcome. Renal abnormalities independent of SRC are possible but are attributed to different pathogenetic mechanisms. Further understanding of pathogenesis of SRC may lead to additional improvement in the therapy of this serious complication.