Acute type A aortic dissection (ATAD) presenting with acute limb ischemia (ALI) has been identified as a predictor of in-hospital mortality. This study examines the outcome of patients presenting with ATAD with ALI. A prospectively collected database was queried for all cases of ATAD repaired between 2002 and 2018 at a tertiary referral center. Patients presenting with ALI were identified. Comparisons between groups with and without lower extremity ALI were made. For continuous variables, univariate two-group comparisons were performed. For categorical variables, univariate two-group comparisons are performed. Statistical significance is set at P < .05. During this period, 378 patients (average age, 56.9 years) underwent ATAD repair. Of these, 62 patients (16.4%) presented with ALI; 35 patients (9.2%) presented with isolated ALI, whereas 27 (7.1%) had concomitant malperfusion in at least one other organ system. Of the 62 patients presenting with ALI, 46 (74.2%) underwent proximal aortic repair alone; 16 (25.8%) also underwent lower extremity vascular intervention (Fig). In the latter part of the study, perfusion of the ischemic limb was accomplished during repair of the ATAD through a side perfusion cannula directly from the bypass circuit (10 patients). There were six amputations (9.7%) performed in the ALI group, two of which had peripheral vascular repairs. Fasciotomies were performed on 18 patients; of these, 5 had concomitant peripheral vascular repairs. Of the 55 patients with ALI surviving past 24 hours, 34 (61%) had resolution of their lower limb ischemia with proximal repair only. The 30-day survival was decreased in patients who presented with any organ malperfusion (P = .012). However, in patients with isolated ALI, there was no significant difference in 30-day mortality (11.4%) compared with the group with no malperfusion (15.7%; P = .5). Sixteen patients underwent peripheral vascular procedures for their limb ischemia, including 10 patients who underwent bypass procedures (7 femoral-femoral, 1 axillary-femoral-femoral, and 2 axillary-femoral), with 1 patient dying within 24 hours. All six patients with adequate follow-up imaging demonstrated asymptomatic occlusion of the bypass graft. In this group, computed tomography angiography showed recanalization of the occluded native arteries. Proximal repair of ATAD resolves most associated ALI. Isolated ALI did not increase 30-day mortality. All patients with follow-up who underwent extra-anatomic bypass developed asymptomatic graft occlusion. This was attributed to competitive flow from the remodeled native arterial system.