Takayasu arteritis (TAK) could cause a stroke or transient ischemic attack (TIA) in young individuals due to inflammatory vascular occlusion or intracerebral hemorrhage. We compared the clinical presentation, angiographic features, longitudinal patterns of disease activity, medical treatments, and survival in 34 TAK patients with stroke/TIA and 157 without stroke/TIA from a single-center retrospective cohort. TAK patients with stroke/TIA were older (p = 0.044) with a greater proportion of males (p = 0.022), more frequent vision loss (odds ratio (OR) for stroke/TIA vs. without stroke TIA 5.21, 95% CI 1.42–19.14), and less frequent pulse or blood pressure inequality (OR 0.43, 95% CI 0.19–0.96) than TAK patients without stroke/TIA. Hata’s angiographic type IIa was more common in TAK patients with stroke/TIA (OR 11.00, 95%CI 2.60–46.58) and type V in TAK patients without stroke/TIA (OR 0.27, 95% CI 0.12–0.58). Cyclophosphamide was used more often in TAK patients with stroke/TIA (p = 0.018). Disease activity at baseline, 6, 12, and 24 months of follow-up was mostly similar for both groups. Risk of mortality was similar in TAK patients with or without stroke/TIA (hazard ratio unadjusted 0.76, 95% CI 0.15–3.99; adjusted for gender, age of disease onset, delay to diagnosis, baseline disease activity, and the number of conventional or biologic/targeted synthetic immunosuppressants used 1.38, 95% CI 0.19–10.20) even after propensity score-matched analyses. Stroke or TIA does not appear to affect survival in TAK patients adversely.