BACKGROUND: Determinants of coronary artery calcification (CAC) prevalence and severity in heterozygous familial hypercholesterolemia (HeFH) remain understudied. The objective of this cross-sectional study was to investigate correlates of CAC in patients with HeFH. METHODS: A CAC score was calculated by a noncontrast computed tomography scan in women (n = 68) and men (n = 78) with genetically defined HeFH. We classified CAC prevalence and severity using 3 categories: CAC score = 0 Agatston Unit (AU), CAC score = 1-100 AU, and CAC score > 100 AU. Information on potential correlates of CAC including familial and personal health history, cardiovascular risk factors, lipid-lowering medication, and lifestyle habits was collected. RESULTS: A total of 95 patients had prevalent CAC. Independent correlates of CAC prevalence and severity included age (odds ratio [OR] per 10 years: 5.06, 95% confidence interval [CI]: 3.19, 7.93, < 0.0001), family history of premature cardiovascular disease (OR: 3.88, 95% CI: 1.71, 8.81,  = 0.001), male sex (OR: 3.40, 95% CI: 1.49, 7.78,  = 0.004), statin use (OR: 15.5, 95% CI: 1.89, 126,  = 0.01), diet quality assessed with the Alternative Healthy Eating Index score (OR per 1 standard deviation: 0.59, 95% CI: 0.39, 0.90,  = 0.01), ever smoking (OR: 3.06, 95% CI: 1.20, 7.81,  = 0.02), receptor-negative genotype (OR: 3.17, 95% CI: 1.16, 8.66,  = 0.02), lipoprotein(a) year-score (OR per 1 standard deviation of log-transformed year-score: 1.53, 95% CI: 0.99, 2.36,  = 0.05). CONCLUSIONS: In individuals with HeFH, age, family history of premature cardiovascular disease, sex, statin use, diet quality, smoking status, the genotype, and lipoprotein(a) concentrations were independently associated with CAC prevalence and severity.