Background: A three-dimensional (3D) cardiovascular magnetic resonance (CMR) vessel wall imaging (VWI) technique based on 3D T1 weighted (T1w) Sampling Perfection with Application-optimized Contrast using different flip angle Evolutions (SPACE) has recently been used as a promising CMR imaging modality for evaluating extracranial and intra-cranial vessel walls. However, this technique is yet to be validated against the current diagnostic imaging standard. We therefore aimed to evaluate the diagnostic performance of 3D CMR VWI in characterizing carotid disease using intra-arterial digital subtraction angiography (DSA) as a reference. Methods: Consecutive patients with at least unilateral > 50% carotid stenosis on ultrasound were scheduled to undergo interventional therapy were invited to participate. The following metrics were measured using 3D CMR VWI and DSA: lumen diameter of the common carotid artery (CCA) and segments C1-C7, stenosis diameter, reference diameter, lesion length, stenosis degree, and ulceration. We assessed the diagnostic sensitivity, specificity, accuracy, and receiver operating characteristic (ROC) curve of 3D CMR VWI, and used Cohen's kappa, the intraclass correlation coefficient (ICC), and Bland-Altman analyses to assess the diagnostic agreement between 3D CMR VWI and DSA. Results: The ICC (all ICCs ≥0.96) and Bland-Altman plots indicated excellent inter-reader agreement in all individual morphologic measurements by 3D CMR VWI. Excellent agreement in all individual morphologic measurements were also found between 3D CMR VWI and DSA. In addition, 3D CMR VWI had high sensitivity (98.4, 97.4, 80.0, 100.0%), specificity (100.0, 94.5, 99.1, 98.0%), and Cohen's kappa (0.99, 0.89, 0.84, 0.96) for detecting stenosis > 50%, stenosis > 70%, ulceration, and total occlusion, respectively, using DSA as the standard. The AUC of 3D CMR VWI for predicting stenosis > 50 and > 70% were 0.998 and 0.999, respectively. Conclusions: The 3D CMR VWI technique enables accurate diagnosis and luminal feature assessment of carotid artery atherosclerosis, suggesting that this imaging modality may be useful for routine imaging workups and provide comprehensive information for both the vessel wall and lumen.