Scleroderma (systemic sclerosis) is a connective tissue disorder which involves skin, gastrointestinal tract, kidneys and lungs with considerable morbidity. Scleroderma renal crisis is a well-known severe form of renal involvement, characterised by the presence of high blood pressure with variable degrees of renal insufficiency. We report a case of scleroderma renal crisis diagnosed on the basis of history of scleroderma, high blood pressure and renal dysfunction. The biopsy confirmed the typical histopathological findings. We discuss herein the management of the case and review the literature.