A rare inherited disorder of copper metabolism in which the body cannot properly absorb and distribute copper, producing severe copper deficiency from infancy. Affected babies develop sparse, kinky, steel-wool hair, fragile connective tissue, weak muscle tone, and progressive neurological damage, often with seizures. The faulty gene impairs a copper-transporting protein, trapping copper in the gut wall. Early treatment with copper injections can improve outcomes if started in the first weeks of life, but the severe classical form remains devastating. Menkes’ syndrome illustrates how a single trace element governs the enzymes that build connective tissue and power the nervous system.