Purpose The aim of this study is to demonstrate the diagnostic effect of VWI in differentiating PACNS from other vasculopathies and its role in post-treatment follow-up in PACNS patients in this study. Methods In this prospective study, we included patients with clinical suspicion of PACNS who presented with new-onset ischemic events and had significant intracranial large vessel stenosis on DSA or MRA. VWI was performed on all patients. The imaging findings and final diagnoses were recorded. Control VWI was performed on patients with PACNS diagnosis after at least 3 months of treatment, and the change in findings was also evaluated. Results Twenty-three patients were included in the study had a median age of 40 (range 12-58). The most common clinical manifestations were focal neurologic deficits. According to the initial clinical evaluation, 10 patients (43.5%) were classified as PACNS and 13 patients (56.5%) as indeterminate for PACNS. After incorporating the VWI findings, the diagnosis of PACNS was confirmed in all clinically diagnosed PACNS patients. Concentric wall thickening and contrast enhancement were statistically significant in the PACNS group (p <0.001). According to concentric thickening and VWE features, sensitivity and specificity in distinguishing PACNS and other vasculopathies were 95.2%, 75% and 95.2%, 68.8%, respectively. Vessel wall enhancement regressed in 7 of 9 patients during a median follow-up period of 8 months (range 5.5-11.5) in PACNS patients who followed up. Conclusion VWI seems a new and useful imaging method in the differential diagnosis of PACNS and might be a useful adjunct for post-treatment follow-up.