Individuals with familial hypercholesterolemia are at markedly increased risk of coronary artery disease (CAD). Accurate risk assessment and appropriate management in this population are therefore crucial. In this review, we summarize findings from several studies that have investigated predictors of CAD in familial hypercholesterolemia (FH). The main categories of predictors include demographic variables and comorbidities, lipid-related parameters, physical findings such as xanthomas, genetic characteristics, and imaging markers of atherosclerosis. We also highlight risk prediction models developed from these previously published findings.