To investigate the association between plasma proprotein convertase subtilisin/kexin type 9 (PCSK9) concentrations, current acute coronary syndrome (ACS), coronary artery disease (CAD) presence, severity and extension and the burden of coronary calcifications in patients with suspected CAD.One hundred and one patients, with or without current ACS, were recruited for this cross-sectional study. CAD presence was defined based on either the presence or absence of at least one significant (≥50%) CAD lesion (SCAD). CAD severity was classified according to the absence of coronary lesions, the presence of non-significant (<50%) CAD (MCAD) or SCAD in at least one major coronary artery. Patients with one, two or three significantly diseased major coronary arteries were defined as 1-SCAD, 2-SCAD and 3-SCAD, respectively. The cumulative length of SCAD lesions and the amount of calcifications in coronary arteries were estimated. Plasma PCSK9 concentrations were higher in patients with SCAD as compared to those without (p = .012). A significant increase in plasma PCSK9 concentrations was observed with greater CAD severity (p = .042). Higher plasma PCSK9 concentrations were found in 3-SCAD patients as compared to either 2-SCAD or 1-SCAD (p < .001). PCSK9 increased with the cumulative length of SCAD lesions and the burden of calcifications (p < .05 for both comparisons). Multivariable adjustment abolished the association between PCSK9 and either CAD presence or severity, but not the association between PCSK9 and the number of significantly diseased vessels, SCAD lesion length and the burden of coronary calcifications. ACS was associated with a borderline significant increase of plasma PCSK9 concentrations among patients not taking statins (p = .05).Circulating PCSK9 concentrations discriminate patients with greater coronary atherosclerotic lesion extension and calcification, and are increased in patients with current ACS.