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Background Familial hypercholesterolaemia (FH) is a primary genetic dyslipidaemia characterized by elevation in serum low-density lipoprotein cholesterol and its deposition in systemic arteries, which causes premature atherosclerosis. Case summary A 10-year-old girl presented with severe symptomatic coronary artery disease. She demonstrated characteristic morphological features of FH. Despite aggressive medical management and lipid-lowering therapy, her symptoms were not relieved and she had dynamic electrocardiogram changes. Coronary angiography showed a distal left main coronary artery lesion along with significant lesions in ostio-proximal and mid-left circumflex artery which were managed by provisional left main coronary artery to left circumflex artery stenting technique, with good immediate- and short-term results and angina relief. Discussion To the best of our knowledge, this is the first reported case of a paediatric patient with FH and acute coronary syndrome treated with percutaneous coronary intervention to left main coronary artery and left circumflex artery using provisional stenting technique. Revascularization strategies for symptomatic coronary artery disease in paediatric patients with FH have multiple unique challenges and remain an unexplored and under-reported subject.

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