Familial hypercholesterolemia is a disease characterized by mutations in the low-density lipoprotein receptor. Total cholesterol levels >500 mg/dL and low-density lipoprotein levels >350 mg/dL are associated with early atherosclerosis. In these patients, the incidence of coronary artery disease is high in the first decade of life, and it often emerges in adolescence. Herein, we present a 14-year-old female with familial hypercholesterolemia who underwent left main coronary artery osteoplasty with a pulmonary autograft due to an incidentally detected osteal stenosis of the left main coronary artery. The patient was discharged without any problems, and no problems were detected during the follow-up.