Objectives Non-A non-B aortic dissection (AD) is a rare and life-threatening medical emergency, and it has been controversial whether it should be managed as type B aortic dissection (TBAD). The study aims to compare in-hospital and follow-up outcomes between patients with non-A non-B AD and those with TBAD treated by endovascular based treatment (EBT). Methods From January 2017 to December 2021, 96 consecutive patients with non-A non-B AD met the inclusion criteria and underwent EBT. Patients with TBAD were matched to patients with non-A non-B AD at a 1:1 ratio using propensity score matching analysis to correct for baseline confounding factors. The primary endpoint was all-cause mortality. Aortic-related events were defined as dissection-related death, aortic rupture, retrograde type A aortic dissection, reintervention, and type Ia endoleak. Results Patients with non-A non-B AD required more TEVAR-related adjunctive procedures compared to TBAD patients during EBT and they required a longer ICU length of stay (36.0 vs 24.0 hours, P < .05) as well as a longer hospitalization (8.0 vs 7.0 days, P < .05) after EBT. There was no statistical difference in overall survival after EBT for patients with TBAD and non-A non-B AD. However, compared to patients with TBAD, non-A non-B AD patients had a higher rate of reintervention and experienced more aortic-related late events during follow-up. Conclusion Patients with non-A non-B acute AD who are treated with EBT do not have higher in-hospital or follow-up mortality rates compared to patients with type B AD. However, there is an increased risk of reintervention and aortic-related late events after the intervention during follow-up.