Intramural hematoma (IMH) of the aorta and penetrating aortic ulcer (PAU) are important variant forms of classic double-barrel aortic dissection in patients presenting with acute aortic syndrome. Recent insights provided by modern high-resolution imaging are currently challenging previous pathophysiologic concepts underlying IMH and PAU, suggesting a close relationship of both entities. Thoracic endovascular aortic repair (TEVAR) offers a less invasive approach to the treatment of affected patients with very encouraging early to midterm results. This review discusses current indication for TEVAR in IMH and PAU patients in the view of an improved understanding of these diseases.