Background: Proprotein Convertase Subtilisin/Kexin type 9 (PCSK9) is associated with hypercholesterolemia and atherosclerotic disease while its inhibition reduces cardiovascular risk. There is some evidence that serum PCSK9 concentrations are higher in patients with acute coronary syndromes compared with those with stable coronary artery disease, which has been attributed to a proatherogenic and prothrombotic state. Objective: This case-control pilot study investigated potential associations of PCSK9 in patients with transient ischemic attack (TIA). Methods: A total of 20 patients with a first-ever atherosclerotic non-cardioembolic TIA and 20 controls of similar age and sex were enrolled. Clinical characteristics, metabolic parameters, including serum PCSK9 within 24 hours from the onset of TIA symptoms were recorded. Results: The serum PCSK9 concentration was higher in TIA patients vs. controls (mean values, 248 ng/mL vs. 196 ng/mL, p = 0.02). In patients with TIA, serum PCSK9 correlated with age (r=0.603, p=0.03), history of coronary artery disease (r=0.515, p=0.020) and ABCD2 score (Age, Blood pressure, Clinical features, symptom Duration, Diabetes – a future stroke prediction tool) (r=0.512, p=0.021). In multivariate analysis, serum PCSK9 was independently associated with higher odds of TIA (1.16 per 10 ng/mL increase, 95% CI 1.01-1.34, p=0.035). Conclusions: Our findings indicate that serum PCSK9 levels are independently associated with atherosclerotic TIA and the risk of future stroke. Further investigation is needed to confirm these findings or to assess the use of PCSK9 as a target for early treatment as well as for secondary stroke prevention.