Introduction: Homozygous familial hypercholesterolemia is a rare condition characterized by markedly elevated levels of low-density lipoprotein (LDL) cholesterol and premature atherosclerotic cardiovascular disease (ASCVD). Patients with homozygous familial hypercholesterolemia often develop clinically significant ASCVD by the second decade of life. Here we report a case of homozygous familial hypercholesterolemia in a young girl who presented with xanthomas at 3 years of age. Case Report: A 3 year-old girl presented for evaluation of skin lesions. Initial exam was notable for crops of tan colored papules on wrists, elbows and posterior ankles. Biopsy showed xanthoma cells with foamy cytoplasm. A lipid panel was significant for LDL 688 mg/dL. Significantly elevated LDL levels along with xanthomas were consistent with the diagnosis of homozygous familial hypercholesterolemia. Family history was notable for dyslipidemia in all paternal and maternal grandparents as well as numerous members of her extended family. Maternal grandmother had a myocardial infarction at 35 years of age. Parents, both in their early 20s, had never been screened for dyslipidemia. The patient was started on rosuvastatin 5 mg nightly. Statin therapy was well tolerated with no noted side effects and LDL decreased to 537 mg/dL on this initial dose. Rosuvastatin dose was increased to 20 mg nightly with corresponding decreased in LDL to 325 mg/dL (53% LDL reduction). Ezetimibe 10 mg daily was added with subsequent decrease in LDL to 147 mg/dL (79% LDL reduction). She has had no GI side effects or myalgias with either medication. Transaminases did increase with initiation of ezetimibe. Aspartate transaminase (AST) peaked at 50 U/L (0-32) and alanine transaminase (ALT) peaked at 50 U/L (0-33). Both AST and ALT normalized without intervention and did not require cessation of medical therapy. Baseline echocardiogram was normal; specifically there was no evidence of valvular or supra-valvular aortic disease. There has been significant regression of xanthomas on follow-up exams as LDL levels have decreased. Discussion: Prompt initiation of treatment directed at lowering LDL levels and reducing other risk factors for cardiovascular disease is the cornerstone of management for patients with homozygous familial hypercholesterolemia. Current guidelines recommend treatment goals for these high-risk patients but achieving these goals is often difficult. Even with early aggressive management, these patients remain at very high risk for premature cardiovascular disease.