Background: Patients with primary biliary cirrhosis (PBC) often present hyperlipidemia, which is a risk factor of incident coronary artery disease (CAD). However, few studies have examined CAD in PBC. Methods: We identified 41 patients who developed CAD amongst 2,675 PBC cases across seven years and selected the PBC patients without CAD as controls according to sex and age. Results: Females dominated in these patients with CAD. The median time from the diagnosis of PBC to the onset of CAD was 44 months. The patient group with CAD had higher median levels of low-density lipoprotein cholesterol (4.68 mmol/L versus 3.52 mmol/L, P=0.036) and higher proportion of cases with hypertension (63.4% versus 19.5%, P<0.001) compared to that without CAD. In the logistic regression, only hypertension (with versus without, P<0.001; OR, 1.597; 95% CI, 1.139-2.053) was selected. Conclusion: PBC patients with hypertension should be monitored carefully due to the risk of incident CAD.