Scleroderma renal crisis is a severe complication of systemic sclerosis characterised by sudden onset of hypertension and acute renal failure, often due to renal microvascular damage. It is clinically significant as it can lead to life-threatening complications if not promptly recognised and treated, typically with ACE inhibitors. A key association is its occurrence in patients with diffuse scleroderma, particularly within the first few years of disease onset.