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Background and purpose Percutaneous transluminal angioplasty and stenting (PTAS) may result in periprocedural embolism. Distal embolic protection devices (dEPDs) are commonly used to mitigate this risk; however, their efficacy in patients with very severe carotid stenosis (VSCS) remains uncertain. In this study we evaluated the periprocedural clinical and MRI outcomes following PTAS in patients with VSCS.Methods We retrospectively included patients with VSCS who underwent PTAS and had periprocedural brain MRI data. Patients were categorized into protected PTAS (P/PTAS) and unprotected PTAS (UP/PTAS) groups according to dEPD use. Recent embolic ischemic lesions (REILs) on diffusion-weighted imaging were quantified within the ipsilateral middle cerebral artery (MCA) territory and categorized by size (<5 or ≥5 mm). The incidence of periprocedural infarction and recurrent ischemic stroke and stent patency were evaluated.Results Among 152 PTAS procedures, 106 were protected and 46 were unprotected. The number of REILs in the ipsilateral MCA territory (median (IQR) 0 (0–2) vs 1 (0–3); P=0.39) and the number of large REILs (≥5 mm) (median (IQR) 0 (0–0) vs 0 (0–1); P=0.14) were compared between groups. With respect to clinical outcomes over the 1-month follow-up period, significant in-stent restenosis occurred in eight of 106 (7.5%) protected procedures and one of 46 (2.2%) unprotected procedures, whereas recurrent ischemic stroke occurred in two of 106 (1.9%) protected procedures and 0 of 46 (0%) unprotected procedures (P=0.28 and P=1.00, respectively).Conclusion In patients with VSCS undergoing PTAS, dEPD should be used when anatomically feasible. When protection cannot be safely deployed, an unprotected anatomy-adapted approach with careful lesion crossing and conservative angioplasty is an acceptable alternative.