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Background: Many guidelines suggest a target LDL-C of Objective: To determine the cardiovascular disease burden and treatment patterns among patients with the ICD-10 code for FH at a tertiary academic center. Methods: A retrospective query was conducted in The University of Pennsylvania Health System (UPHS) using the ICD-10 code, E78.01. Results: The query returned 1417 FH patients (703 seen in the lipid clinic, 714 seen elsewhere in UPHS) identified by E78.01. Among all patients, median age at initiation of lipid-lowering therapy was 52 years, and median age at FH diagnosis was 55 years. Prevalent coronary heart disease was reported in 30% (425 of 1417) of all patients, and 27% (382 of 1417) exhibited a family history of cardiovascular disease. Median untreated low-density lipoprotein cholesterol (LDL-C) was 197 mg/dL and median treated LDL-C was 112 mg/dL. Among all patients, 35% (495 of 1417) were taking high-intensity statin therapy, 24% (340 of 1417) were taking PCSK9 inhibitor (PCSK9i) therapy, and 29% (410 of 1417) were taking ezetimibe. Providers in the Penn Lipid Clinic prescribed approximately 71% (557 of 785) of all high-intensity statin and PCSK9i therapy to FH patients of whom 50% (392 of 785) achieved an LDL-C of Conclusions: The ICD-10 code for FH identifies a cohort of patients with a phenotype consistent with FH. A large minority of FH patients are not taking high-intensity statin or PCSK9i therapy, especially those who have not seen a provider in a dedicated lipid clinic. These findings highlight a way to monitor the clinical management of FH patients while explaining need for education about FH in an academic center.