Background and aim: Little is known about the cardioprotective potential of a healthy lifestyle in familial hypercholesterolemia (FH). The objective of this study was to evaluate the relationship between lifestyle and cardiovascular risk factors in adults with FH. Methods and results: This cross-sectional study leveraged data from the CARTaGENE Quebec population-based cohort (Canada). Participants with FH were identified using the validated Simplified Canadian Definition for FH. A healthy lifestyle score (HLS), ranging from 0 to 5, was calculated per adherence to 5 lifestyle habits: 1) not smoking; 2) being physically active (≥150 min/week of moderate or vigorous physical activity); 3) eating a healthy diet (Alternate Healthy Eating Index ≥50%); 4) having a light to moderate alcohol consumption (men: 1–30 g/day; women: 1–15 g/day); and 5) sleeping 7–8 h/day. Among the 122 included individuals (women, n = 78; men, n = 44; mean age ± SD: 57.3 ± 6.7 years), 92 (75.4%) had a HLS ≤3/5, while only 5 (4.1%) had a HLS of 5/5. After adjustments for sex, age, body mass index, and lipid-lowering medication use, we found no evidence of an association between the HLS and concentrations of LDL-cholesterol (β = 0.04, 95% CI = −0.08, 0.15 mmol/L; P = 0.54). However, the HLS was favorably associated with HbA1c levels (β = −0.07, 95% CI = −0.13, −0.01%; P = 0.02), and statistical trends suggested favorable associations with HDL-cholesterol (β = 0.06, 95% CI = −0.02, 0.14 mmol/L; P = 0.06) and waist circumference (β = −2.22, 95% CI = −4.62, 0.17 cm; P = 0.07). Conclusion: This study suggests that a healthy lifestyle is favorably associated with CVD risk factors in adults with FH.