Homozygous familial hypercholesterolemia (HFH), resulting from mutation of the low-density lipoprotein (LDL) receptor gene with subsequent defective catabolism of the LDL cholesterol, leads to premature atherosclerosis and coronary artery disease. We report a successful urgent stent implantation in a 7-year-old patient with HFH and a severe left main coronary artery stenosis, who presented critical acute myocardial ischemia immediately after left coronary angiography. Percutaneous transluminal coronary angiography with stent implantation allowed for rapid coronary flow reestablishment without sequelae. Aggressive lipid-lowering therapy with high doses statins and LDL-apheresis was maintained, together with lifelong antiaggregant acetylsalicylic treatment with good short-term patency of the stent at 1-year follow-up. In conclusion, coronary artery stent implantation may be considered as a valid rescue therapeutic alternative for pediatric patients with HFH and severe ostial coronary stenosis in case of emergency.