Isolated abdominal aortic dissection (IAAD) is a rare condition with the primary entry tear located in the abdominal aorta, which might occur spontaneously, or be the consequence of trauma and iatrogenic events as well. Male, hypertension and concomitant aortic aneurysm may be high risk factors for IAAD. The frequent presentations at onset are abdominal pain, back pain, and intermittent claudication. Therapeutic choices for IAAD include medical treatment, conventional open surgical repair, endovascular repair and hybrid surgery. Recently, endovascular repair has increasingly become the most preferred treatment option. In this article, the authors address the clinical features and management advances of IAAD.