Idiopathic copper-associated cirrhosis

**Definition:** Idiopathic copper-associated cirrhosis is a rare copper-overload liver disease characterised by a rapidly progressive liver cirrhosis from the first few years of life leading to hepatic insufficiency and harbouring a specific pathological aspect: pericellular fibrosis, inflammatory infiltration, hepatocyte necrosis, absence of steatosis, poor regeneration and histochemical copper staining.