Acute aortic dissection (AAD) is a severe and potentially fatal disease which requires rapid diagnosis and appropriate treatment in order to improve survival. The variable and sometimes non-specific presentation of this rare condition makes it a continued diagnostic challenge for even the best of clinicians. Multiple clinical factors have been studied for their role in increasing the risk of AAD. While the complete pathophysiology of the condition is not known, contemporary research has shed light on many important factors that might lead to the development of aortic aneurysms and AAD. Despite continued research in this area, there is scant research looking at gender-related differences in the epidemiology, presentation, treatment and outcomes of patients with AAD. This review first revisits lessons about gender-related differences seen in various cardiovascular diseases, and then critically examines the data on gender-related differences in AAD diagnosis, management and outcomes. A summary of the pathophysiology and possible reasons for gender-specific differences in the development of aneurysms and dissections is also provided.